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1. Tupikowski W, Bednarek-Tupikowska G, Florczak A: [Adrenocortical carcinoma and its treatment]. Postepy Hig Med Dosw (Online); 2004 Feb 26;58:27-36
Hazardous Substances Data Bank. MITOTANE .

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Adrenocortical carcinoma and its treatment].
  • Adrenocortical carcinoma is a rare tumor with an annual incidence of 1 to 2 cases per million people.
  • Most adrenocortical neoplasms are hormone functional.
  • The rapid onset of Cushing 's syndrome, with its virilizing features, is characteristic of this cancer.
  • Adrenal tumors are often detected at an advanced stage.
  • Complete surgical resection is the only curative treatment for adrenal cancer.
  • Treatment also includes chemotherapy, especially with mitotane, usually in combination with doxorubicin, etoposide, and cisplatin.
  • Results of treatment are not satisfying, so adjuvant multicenter trials are still underway.

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  • (PMID = 15069374.001).
  • [ISSN] 1732-2693
  • [Journal-full-title] Postepy higieny i medycyny doswiadczalnej (Online)
  • [ISO-abbreviation] Postepy Hig Med Dosw (Online)
  • [Language] POL
  • [Publication-type] English Abstract; Journal Article; Review
  • [Publication-country] Poland
  • [Chemical-registry-number] 6PLQ3CP4P3 / Etoposide; 78E4J5IB5J / Mitotane; 80168379AG / Doxorubicin; Q20Q21Q62J / Cisplatin
  • [Number-of-references] 48
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2. Nishida S, Itoh N, Sasao T, Masumori N, Taguchi K, Tsukamoto T: Adrenocortical carcinoma: retrospective study of 14 patients experienced at a single institution over 34 years. Int J Urol; 2007 Jul;14(7):581-4
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Adrenocortical carcinoma: retrospective study of 14 patients experienced at a single institution over 34 years.
  • OBJECTIVE: To review clinical outcome of patients with adrenocortical carcinoma experienced at a single institute over 34 years.
  • Two patients were classified as having stage II disease, seven as stage III and five as stage IV.
  • The median survival periods were 2 months in patients with stage IV and 108 months in those with stages II and III (P = 0.136).
  • Mitotane treatment in the adjuvant setting did not clearly affect the clinical courses of patients without metastasis.
  • However, the treatment was effective for metastasis that was repeatedly developed as late recurrence in one patient.
  • Three patients with metastasis at diagnosis received combination chemotherapy with etoposide, doxorubicin and cisplatin (EDP) with or without mitotane treatment, to which lung metastasis completely responded in one patient.
  • CONCLUSIONS: Adrenocortical carcinoma is a rare disease but frequently recurs.
  • The best chance of survival may be achieved by early detection and complete surgical removal.
  • There may be patients who possibly benefit from mitotane treatment with or without EDP, although this remains to be conclusively determined.
  • [MeSH-major] Adrenal Cortex Neoplasms. Adrenocortical Carcinoma
  • [MeSH-minor] Adult. Aged. Child, Preschool. Female. Humans. Male. Middle Aged. Retrospective Studies. Time Factors

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  • (PMID = 17645596.001).
  • [ISSN] 0919-8172
  • [Journal-full-title] International journal of urology : official journal of the Japanese Urological Association
  • [ISO-abbreviation] Int. J. Urol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Australia
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3. Terzolo M, Angeli A, Fassnacht M, Daffara F, Tauchmanova L, Conton PA, Rossetto R, Buci L, Sperone P, Grossrubatscher E, Reimondo G, Bollito E, Papotti M, Saeger W, Hahner S, Koschker AC, Arvat E, Ambrosi B, Loli P, Lombardi G, Mannelli M, Bruzzi P, Mantero F, Allolio B, Dogliotti L, Berruti A: Adjuvant mitotane treatment for adrenocortical carcinoma. N Engl J Med; 2007 Jun 7;356(23):2372-80
Hazardous Substances Data Bank. MITOTANE .

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Adjuvant mitotane treatment for adrenocortical carcinoma.
  • BACKGROUND: Adrenocortical carcinoma is a rare neoplasm characterized by a high risk of recurrence after radical resection.
  • Whether the use of mitotane is beneficial as an adjuvant treatment has been controversial.
  • METHODS: We performed a retrospective analysis involving 177 patients with adrenocortical cancer who had undergone radical surgery at 8 centers in Italy and 47 centers in Germany between 1985 and 2005.
  • Adjuvant mitotane was administered to 47 Italian patients after radical surgery (mitotane group), whereas 55 Italian patients and 75 German patients (control groups 1 and 2, respectively) did not receive adjuvant treatment after surgery.
  • RESULTS: Baseline features in the mitotane group and the control group from Italy were similar; the German patients were significantly older (P=0.03) and had more stage I or II adrenocortical carcinomas (P=0.02) than did patients in the mitotane group.
  • Multivariate analysis indicated that mitotane treatment had a significant advantage for recurrence-free survival.
  • CONCLUSIONS: Adjuvant mitotane may prolong recurrence-free survival in patients with radically resected adrenocortical carcinoma.
  • [MeSH-major] Adrenal Cortex Neoplasms / drug therapy. Adrenocortical Carcinoma / drug therapy. Antineoplastic Agents, Hormonal / therapeutic use. Mitotane / therapeutic use
  • [MeSH-minor] Chemotherapy, Adjuvant. Humans. Multivariate Analysis. Neoplasm Recurrence, Local / epidemiology. Neoplasm Recurrence, Local / prevention & control. Retrospective Studies. Survival Analysis


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4. Wängberg B, Khorram-Manesh A, Jansson S, Nilsson B, Nilsson O, Jakobsson CE, Lindstedt S, Odén A, Ahlman H: The long-term survival in adrenocortical carcinoma with active surgical management and use of monitored mitotane. Endocr Relat Cancer; 2010 Mar;17(1):265-72
Hazardous Substances Data Bank. MITOTANE .

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  • [Title] The long-term survival in adrenocortical carcinoma with active surgical management and use of monitored mitotane.
  • Adrenocortical carcinoma (ACC) is a rare tumour disease with sinister prognosis also after attempts to radical surgery; better prognosis is seen for low-stage tumours.
  • Adjuvant treatment with the adrenolytic drug mitotane has been attempted, but not proven to prevent from recurrence.
  • The drug may offer survival advantage in case of recurrence.
  • The aim of this single-centre study (1979-2007) of 43 consecutive patients was to evaluate the long-term survival after active surgical treatment combined with monitored mitotane (to reduce side effects of the drug).
  • The series is unique, since all patients were offered a period of mitotane as adjuvant or palliative treatment; six patients refused mitotane.
  • Despite a high proportion of high-stage tumours (67%), the complete resection rate was high (77%).
  • The disease-specific 5-year survival was high (64.1%); very high for patients with low-stage tumours without evident relation to mitotane levels.
  • Patients with high-stage tumours had a clear survival advantage with mitotane levels above a threshold of 14 mg/l in serum.
  • The hazard ratio for patients with high mitotane levels versus all patients indicates a significant effect of the drug.
  • The results indicate that adjuvant mitotane may be the standard of care for patients with high-stage ACC after complete resection.
  • [MeSH-major] Adrenal Cortex Neoplasms / drug therapy. Adrenal Cortex Neoplasms / mortality. Adrenal Cortex Neoplasms / surgery. Adrenocortical Carcinoma / drug therapy. Adrenocortical Carcinoma / mortality. Adrenocortical Carcinoma / surgery. Mitotane / therapeutic use
  • [MeSH-minor] Adult. Aged. Aged, 80 and over. Algorithms. Antineoplastic Agents, Hormonal / therapeutic use. Combined Modality Therapy. Endocrine Surgical Procedures / methods. Female. Humans. Male. Middle Aged. Monitoring, Physiologic. Survival Analysis. Survivors / statistics & numerical data. Time Factors. Young Adult

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  • (PMID = 20026647.001).
  • [ISSN] 1479-6821
  • [Journal-full-title] Endocrine-related cancer
  • [ISO-abbreviation] Endocr. Relat. Cancer
  • [Language] eng
  • [Publication-type] Evaluation Studies; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antineoplastic Agents, Hormonal; 78E4J5IB5J / Mitotane
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5. Ohwada S, Izumi M, Kawate S, Hamada K, Toya H, Togo N, Horiguchi J, Koibuchi Y, Takahashi T, Yamada M: Surgical outcome of stage III and IV adrenocortical carcinoma. Jpn J Clin Oncol; 2007 Feb;37(2):108-13
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Surgical outcome of stage III and IV adrenocortical carcinoma.
  • BACKGROUND: Adrenocortical carcinoma (ACC) is a rare tumor usually diagnosed at an advanced stage on invasion of or adherence to adjacent organs.
  • We report surgical outcome of stage III and IV ACCs.
  • METHODS: ACCs from seven patients at clinical stage II (n = 1), III (n = 4), or IV (n = 2) were resected.
  • RESULTS: The pathological stage was stage III in five patients and stage IV in two patients.
  • The estimated 3-year disease-free and overall survivals for stage III were 20% and 40%, respectively, with a median follow-up of 32 months (range, 11-58).
  • The 3-year disease-free and overall survivals for stage III and IV were 14.3% and 28.6%, respectively.
  • The mean disease-free survival time was 18.6 +/- 6.7 months (95% CI: 5.4-31.8).
  • The mean survival after recurrence was 19.0 +/- 3.3 months (95% CI: 12.6-25.5), and the 50% survival was 18.4 months with mitotan and cytotoxic drug therapy.
  • CONCLUSIONS: Resection for stage III, IV ACCs affords the possibility of negative margins, acceptable peri-operative morbidity and mortality, and prolongs survival in selected patients.
  • [MeSH-major] Adrenal Cortex Neoplasms / surgery. Adrenocortical Carcinoma / surgery

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  • (PMID = 17277000.001).
  • [ISSN] 1465-3621
  • [Journal-full-title] Japanese journal of clinical oncology
  • [ISO-abbreviation] Jpn. J. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Japan
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6. Zimmerman MA, Trotter JF, Wachs M, Bak T, Campsen J, Skibba A, Kam I: Sirolimus-based immunosuppression following liver transplantation for hepatocellular carcinoma. Liver Transpl; 2008 May;14(5):633-8
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Sirolimus-based immunosuppression following liver transplantation for hepatocellular carcinoma.
  • In combination with calcineurin inhibitors (CNIs), SRL may reduce the incidence of acute rejection and lower overall required drug levels.
  • This study sought to quantify long-term outcome following OLT in patients with cirrhosis and concomitant hepatocellular carcinoma (HCC) who were treated with an SRL-based regimen as a primary therapy.
  • From January 2000 to June 2007, 97 patients underwent OLT for end-stage liver disease and HCC at the University of Colorado Health Sciences Center.
  • The 2 treatment groups were compared with respect to the following variables: age, gender, tumor stage by explant, grade, size, presence of vascular invasion, focality, Child's class, baseline creatinine, and warm and cold ischemic times.
  • The 2 groups were comparable by all factors save for cold ischemic time, which was significantly longer in the CNI-treated group.
  • Conversely, survival in patients treated with CNIs exclusively at the same time intervals was 83% and 62%.
  • Cumulatively, these data suggest a potential survival benefit with SRL-based therapy in patients undergoing OLT for end-stage liver disease and concomitant malignancy.
  • [MeSH-major] Carcinoma, Hepatocellular / surgery. Graft Rejection / prevention & control. Immunosuppressive Agents / therapeutic use. Liver Failure / surgery. Liver Neoplasms / surgery. Liver Transplantation. Neoplasm Recurrence, Local / prevention & control. Sirolimus / therapeutic use
  • [MeSH-minor] Adrenal Cortex Hormones / therapeutic use. Calcineurin Inhibitors. Cyclosporine / therapeutic use. Drug Therapy, Combination. Female. Humans. Kaplan-Meier Estimate. Kidney Diseases / chemically induced. Male. Middle Aged. Mycophenolic Acid / analogs & derivatives. Mycophenolic Acid / therapeutic use. Proportional Hazards Models. Retrospective Studies. Risk Assessment. Tacrolimus / therapeutic use. Time Factors. Treatment Outcome

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  • (PMID = 18324656.001).
  • [ISSN] 1527-6473
  • [Journal-full-title] Liver transplantation : official publication of the American Association for the Study of Liver Diseases and the International Liver Transplantation Society
  • [ISO-abbreviation] Liver Transpl.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Adrenal Cortex Hormones; 0 / Calcineurin Inhibitors; 0 / Immunosuppressive Agents; 83HN0GTJ6D / Cyclosporine; 9242ECW6R0 / mycophenolate mofetil; HU9DX48N0T / Mycophenolic Acid; W36ZG6FT64 / Sirolimus; WM0HAQ4WNM / Tacrolimus
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7. Kasperlik-Zaluska AA: Clinical results of the use of mitotane for adrenocortical carcinoma. Braz J Med Biol Res; 2000 Oct;33(10):1191-6
Hazardous Substances Data Bank. MITOTANE .

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Clinical results of the use of mitotane for adrenocortical carcinoma.
  • The drug has been used for 40 years in the treatment of adrenocortical carcinoma, mainly its regional and metastatic stage, as an adjuvant to surgical resection of the tumor.
  • In the medical literature there are controversial opinions about its efficacy for the treatment of adrenocortical carcinoma.
  • We have administered this drug in all cases of microscopically confirmed adrenocortical carcinoma, irrespectively of stage at the time of surgery, for fear of a false too optimistic classification.
  • In our series of 82 patients with adrenocortical carcinoma, 59 patients have been treated with mitotane, 32 of them immediately after surgery, and 27 with a delay of 2 to 24 months.
  • All patients were simultaneously given replacement therapy.
  • Furthermore, we used mitotane with good results in Cushing's syndrome of non-malignant origin as pre-treatment before surgery or in long-term treatment for patients with poor tolerance of other adrenal inhibitors.

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  • (PMID = 11004719.001).
  • [ISSN] 0100-879X
  • [Journal-full-title] Brazilian journal of medical and biological research = Revista brasileira de pesquisas medicas e biologicas
  • [ISO-abbreviation] Braz. J. Med. Biol. Res.
  • [Language] ENG
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Brazil
  • [Chemical-registry-number] 0 / Antineoplastic Agents, Hormonal; 78E4J5IB5J / Mitotane
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8. Münstedt K, Borces D, Bohlmann MK, Zygmunt M, von Georgi R: Glucocorticoid administration in antiemetic therapy: is it safe? Cancer; 2004 Oct 1;101(7):1696-702
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Glucocorticoid administration in antiemetic therapy: is it safe?
  • BACKGROUND: Although glucocorticoids are often used in cancer therapy, in particular to enhance the effectiveness of antiemetic therapy, they have been associated with impaired tumor apoptosis and an increased frequency of metastases in some reports.
  • The current study aimed to determine whether glucocorticoid treatment had an adverse effect on outcomes in patients with ovarian carcinoma.
  • METHODS: Records of patients with ovarian carcinoma who were scheduled to receive at least six courses of systemic chemotherapy were reviewed.
  • Patients were grouped into those who had or had not received corticosteroid medication as a part of general antiemetic prophylaxis before chemotherapy, and details of hematologic parameters during treatment and disease recurrence-free and overall survival were recorded.
  • RESULTS: Altogether, 245 patients with ovarian carcinoma had received chemotherapy.
  • Of these, 62 had been given concurrent glucocorticoid treatment and 183 had not.
  • The two patient groups were well balanced with respect to disease stage and other prognostic factors.
  • Patients who received glucocorticoid treatment had significantly higher leukocyte values in the days immediately after chemotherapy, higher nadir leukocyte values, and higher counts before subsequent courses of chemotherapy (P < 0.01; Levene test, t test) compared with patients who did not receive glucocorticoid treatment.
  • As a result, the initial treatment targets were achieved significantly more often in the glucocorticoid group (P = 0.007; chi-square test).
  • CONCLUSIONS: There was no evidence that glucocorticoid treatment had a negative effect on outcomes in these patients.
  • [MeSH-major] Adrenal Cortex Hormones / adverse effects. Antiemetics / adverse effects. Ovarian Neoplasms / drug therapy
  • [MeSH-minor] Adult. Aged. Dexamethasone / administration & dosage. Dexamethasone / adverse effects. Female. Humans. Leukocyte Count. Middle Aged. Prognosis. Retrospective Studies. Treatment Outcome

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  • [Copyright] (c) 2004 American Cancer Society.
  • [CommentIn] Cancer. 2005 Jun 15;103(12):2656; author reply 2656-7 [15856471.001]
  • (PMID = 15468188.001).
  • [ISSN] 0008-543X
  • [Journal-full-title] Cancer
  • [ISO-abbreviation] Cancer
  • [Language] eng
  • [Publication-type] Clinical Trial; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Adrenal Cortex Hormones; 0 / Antiemetics; 7S5I7G3JQL / Dexamethasone
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9. Borrelli D, Bergamini C, Borrelli A, Reddavide S, Lassig R, Valeri A: [Surgical strategy in the treatment of adrenal cortex cancer. Expanded and repeated interventions]. Ann Ital Chir; 2003 May-Jun;74(3):311-7
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Surgical strategy in the treatment of adrenal cortex cancer. Expanded and repeated interventions].
  • [Transliterated title] La strategia chirurgica nel trattamento del carcinoma cortico surrenalico. Gli interventi allargati ed iterativi.
  • AIM OF THE STUDY: To analyze our patients affected by adreno-cortical carcinoma (ACC) considering in particular the therapeutical approach in case of local recurrence or metastasis, and to compare our results with those from literature.
  • Adjuvant treatment was administered in 17 patients, 4 out of which were re-operated.
  • RESULTS: Only one patient died in the perioperative period for hyperacute adrenal failure.
  • Local recurrence or metastatic disease was observed in 27 patients, out of which only 9 were eventually surgically treated, once or more times.
  • Interestingly, one patient who has been re-operated three times, is still alive and disease-free after 7 years from the first recurrence.
  • CONCLUSIONS: According with data from literature, we conclude that surgical therapy of recurring local or metastatic ACC is up to now the best treatment, independently from the original stage of the disease.
  • Controversies still remain about the utility of adjuvant chemotherapy in the primary and the recurrent disease.
  • [MeSH-major] Adrenal Cortex Neoplasms / surgery. Adrenalectomy / methods
  • [MeSH-minor] Adolescent. Adult. Aged. Chemotherapy, Adjuvant. Child. Child, Preschool. Combined Modality Therapy. Disease-Free Survival. Female. Follow-Up Studies. Humans. Laparoscopy. Life Tables. Lymph Node Excision. Male. Middle Aged. Mitotane / therapeutic use. Neoplasm Invasiveness. Neoplasm Metastasis. Neoplasm Recurrence, Local. Palliative Care. Reoperation. Retrospective Studies. Survival Analysis. Treatment Outcome

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  • (PMID = 14677288.001).
  • [ISSN] 0003-469X
  • [Journal-full-title] Annali italiani di chirurgia
  • [ISO-abbreviation] Ann Ital Chir
  • [Language] ita
  • [Publication-type] English Abstract; Journal Article; Review
  • [Publication-country] Italy
  • [Chemical-registry-number] 78E4J5IB5J / Mitotane
  • [Number-of-references] 26
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10. Tucci S Jr, Martins AC, Suaid HJ, Cologna AJ, Reis RB: The impact of tumor stage on prognosis in children with adrenocortical carcinoma. J Urol; 2005 Dec;174(6):2338-42, discussion 2342
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] The impact of tumor stage on prognosis in children with adrenocortical carcinoma.
  • PURPOSE: We evaluated treatment outcomes in children with adrenocortical carcinoma.
  • Children with incomplete excision of the tumor and/or stage IV disease received adjuvant chemotherapy.
  • RESULTS: Ultrasonography, computerized tomography and magnetic resonance imaging exhibited specificity of 100% in the diagnosis of vascular invasion, and sensitivity of 50%, 66% and 100%, respectively.
  • Patient age, tumor stage or size and vascular invasion were associated with survival in univariate analysis.
  • Tumor stage was the only independent factor associated with survival in multivariate analysis.
  • The overall 5-year survival rates according to tumor stage were 100% in stage I, 85% in stage II, 40% in stage III and 0% in stage IV.
  • A total of 10 children with stage IV disease treated with chemotherapy died within a median of 6 months.
  • CONCLUSIONS: Tumor stage was the most relevant prognostic factor for children with adrenocortical carcinoma.
  • [MeSH-major] Adrenal Cortex Neoplasms / diagnosis. Adrenocortical Carcinoma / diagnosis
  • [MeSH-minor] Adolescent. Cardiopulmonary Bypass. Child. Child, Preschool. Female. Follow-Up Studies. Humans. Infant. Magnetic Resonance Imaging. Male. Multivariate Analysis. Neoplasm Invasiveness. Neoplasm Recurrence, Local / epidemiology. Neoplasm Recurrence, Local / etiology. Neoplasm Staging. Prognosis. Retrospective Studies. Sensitivity and Specificity. Survival Analysis. Thrombectomy. Tomography, X-Ray Computed. Treatment Outcome

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  • (PMID = 16280838.001).
  • [ISSN] 0022-5347
  • [Journal-full-title] The Journal of urology
  • [ISO-abbreviation] J. Urol.
  • [Language] eng
  • [Publication-type] Evaluation Studies; Journal Article
  • [Publication-country] United States
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11. Allolio B, Hahner S, Weismann D, Fassnacht M: Management of adrenocortical carcinoma. Clin Endocrinol (Oxf); 2004 Mar;60(3):273-87
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Management of adrenocortical carcinoma.
  • Adrenocortical carcinoma (ACC) is a rare neoplasm with poor prognosis.
  • In addition, computed tomography (CT) typically demonstrates an inhomogeneous adrenal lesion with irregular margins and variable enhancement of solid components after intravenous contrast media.
  • Complete tumour removal (R0 resection) offers by far the best chance for long-term survival and therefore surgery is the treatment of choice in stage I-III ACC.
  • Thus adjuvant treatment options need to be evaluated in high-risk patients (e.g. radiation therapy of the tumour bed and/or chemotherapy).
  • In metastatic disease (stage IV ACC) not amenable to surgery mitotane (o,p'DDD) remains the first-line therapy.
  • Drug monitoring is needed for effective treatment aiming at concentrations between 14 and 20 mg/l.
  • Patients not responding to mitotane may benefit from cytotoxic chemotherapy (23% partial remissions, 4% complete remissions).
  • Only large prospective multicentre trials comparing different treatment options will allow to make systematic progress in the management of ACC.
  • [MeSH-major] Adrenal Cortex Neoplasms / therapy. Carcinoma / therapy
  • [MeSH-minor] Antineoplastic Agents / therapeutic use. Cytokines / therapeutic use. Humans. Magnetic Resonance Imaging. Neoplasm Staging. Prognosis. Tomography, X-Ray Computed

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  • (PMID = 15008991.001).
  • [ISSN] 0300-0664
  • [Journal-full-title] Clinical endocrinology
  • [ISO-abbreviation] Clin. Endocrinol. (Oxf)
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Cytokines
  • [Number-of-references] 154
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12. Gonzalez RJ, Tamm EP, Ng C, Phan AT, Vassilopoulou-Sellin R, Perrier ND, Evans DB, Lee JE: Response to mitotane predicts outcome in patients with recurrent adrenal cortical carcinoma. Surgery; 2007 Dec;142(6):867-75; discussion 867-75
Hazardous Substances Data Bank. MITOTANE .

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Response to mitotane predicts outcome in patients with recurrent adrenal cortical carcinoma.
  • BACKGROUND: Adrenal cortical carcinoma (ACC) is a rare disease in which recurrence after surgery is common.
  • Mitotane is the primary systemic treatment for recurrent ACC; data are limited regarding the impact of mitotane on recurrent ACC.
  • Higher stage at presentation (P = .002) and tumor cortisol production (P = .007) were associated with a worse OS.
  • Mitotane should be considered in most patients with recurrent ACC, including as preoperative therapy for those with recurrent disease considered for surgical resection.
  • [MeSH-major] Adrenal Cortex Neoplasms / drug therapy. Adrenal Cortex Neoplasms / surgery. Antineoplastic Agents, Hormonal / therapeutic use. Mitotane / therapeutic use. Neoplasm Recurrence, Local / drug therapy. Neoplasm Recurrence, Local / surgery
  • [MeSH-minor] Adolescent. Adult. Aged. Child. Child, Preschool. Combined Modality Therapy. Disease-Free Survival. Female. Follow-Up Studies. Humans. Kaplan-Meier Estimate. Male. Middle Aged. Predictive Value of Tests. Prognosis

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  • (PMID = 18063070.001).
  • [ISSN] 1532-7361
  • [Journal-full-title] Surgery
  • [ISO-abbreviation] Surgery
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents, Hormonal; 78E4J5IB5J / Mitotane
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13. Xiao ZJ, Li CL: [Combined therapy of advanced adrenal cortical adenocarcinoma]. Zhonghua Yi Xue Za Zhi; 2010 Aug 10;90(30):2123-5

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Combined therapy of advanced adrenal cortical adenocarcinoma].
  • OBJECTIVE: To analyze the clinical efficacy of combined therapy in the treatment of advanced adrenal cortical adenocarcinoma.
  • METHODS: The clinical data of 12 cases with advanced adrenal cortical adenocarcinoma at our hospital from 1986 - 2006 were analyzed.
  • All cases were treated with chemotherapy or chemotherapy pulse post-operative radiotherapy.
  • Pathological diagnosis was all of adrenal cortical adenocarcinoma.
  • According to the staging criteria of Jacques and Brennan, all 12 cases were of IV stage.
  • The median survival time was 14 months and median progression-free survival time 9 months.
  • CONCLUSION: Combined therapy of adrenal cortical adenocarcinoma is effective to prolong the patient lifespan.
  • Making an early diagnosis and offering a novel therapy yield a better outcome.
  • [MeSH-major] Adrenal Cortex Neoplasms / pathology. Adrenal Cortex Neoplasms / therapy. Adrenocortical Carcinoma / pathology. Adrenocortical Carcinoma / therapy
  • [MeSH-minor] Adult. Combined Modality Therapy. Female. Humans. Male. Middle Aged. Neoplasm Staging. Retrospective Studies. Treatment Outcome

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  • (PMID = 21029628.001).
  • [ISSN] 0376-2491
  • [Journal-full-title] Zhonghua yi xue za zhi
  • [ISO-abbreviation] Zhonghua Yi Xue Za Zhi
  • [Language] chi
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] China
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14. Ahlman H, Khorram-Manesh A, Jansson S, Wängberg B, Nilsson O, Jacobsson CE, Lindstedt S: Cytotoxic treatment of adrenocortical carcinoma. World J Surg; 2001 Jul;25(7):927-33
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Cytotoxic treatment of adrenocortical carcinoma.
  • Adrenocortical carcinoma (ACC) is a rare, aggressive tumor that is often detected in an advanced stage.
  • Medical treatment with the adrenotoxic drug mitotane has been used for decades, but critical prospective trials on its role in residual disease or as an adjuvant agent after surgical resection are still lacking.
  • The concept of a critical threshold plasma level of the drug must be confirmed in controlled studies.
  • In case of cortisol or other steroid overproduction, several drugs (e.g., ketoconazole or aminoglutethimide) may be used.
  • Chemotherapy with single agents (e.g., doxorubicin or cisplatin) have been disappointing, with low response rates (< 30%) and a short response duration.
  • Chemotherapy with multiple agents has been tested in smaller series and has resulted in significant side effects.
  • When mitotane-based therapies are used, monitored drug levels are mandatory.
  • [MeSH-major] Adrenal Cortex Neoplasms / drug therapy. Adrenocortical Carcinoma / drug therapy. Antineoplastic Agents / therapeutic use
  • [MeSH-minor] Cisplatin / therapeutic use. Doxorubicin / therapeutic use. Etoposide / therapeutic use. Humans. Mitotane / therapeutic use

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  • (PMID = 11572034.001).
  • [ISSN] 0364-2313
  • [Journal-full-title] World journal of surgery
  • [ISO-abbreviation] World J Surg
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 6PLQ3CP4P3 / Etoposide; 78E4J5IB5J / Mitotane; 80168379AG / Doxorubicin; Q20Q21Q62J / Cisplatin
  • [Number-of-references] 67
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15. Soga H, Takenaka A, Ooba T, Nakano Y, Miyake H, Takeda M, Tanaka K, Hara I, Fujisawa M: A twelve-year experience with adrenal cortical carcinoma in a single institution: long-term survival after surgical treatment and transcatheter arterial embolization. Urol Int; 2009;82(2):222-6

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] A twelve-year experience with adrenal cortical carcinoma in a single institution: long-term survival after surgical treatment and transcatheter arterial embolization.
  • INTRODUCTION: We retrospectively analyzed 6 cases of adrenal cortical carcinoma (ACC) treated during a 12-year period at a single institution.
  • Three patients were clinically classified as stage II, 2 as stage III and 1 as stage IV with bone metastasis.
  • Two patients without reoperation for metastases died, and others with radical treatments such as surgery, chemotherapy, transcatheter arterial embolization and/or radiation remain alive.
  • [MeSH-major] Adrenal Cortex Neoplasms / therapy. Adrenalectomy. Adrenocortical Carcinoma / therapy. Chemoembolization, Therapeutic
  • [MeSH-minor] Adult. Aged. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Bone Neoplasms / secondary. Bone Neoplasms / therapy. Female. Humans. Japan. Liver Neoplasms / secondary. Liver Neoplasms / therapy. Male. Middle Aged. Neoplasm Staging. Reoperation. Retrospective Studies. Survival Analysis. Time Factors. Tomography, X-Ray Computed. Treatment Outcome

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  • [Copyright] Copyright 2009 S. Karger AG, Basel.
  • (PMID = 19322014.001).
  • [ISSN] 1423-0399
  • [Journal-full-title] Urologia internationalis
  • [ISO-abbreviation] Urol. Int.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Switzerland
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16. Koschker AC, Fassnacht M, Hahner S, Weismann D, Allolio B: Adrenocortical carcinoma -- improving patient care by establishing new structures. Exp Clin Endocrinol Diabetes; 2006 Feb;114(2):45-51
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Adrenocortical carcinoma -- improving patient care by establishing new structures.
  • BACKGROUND: Adrenocortical carcinoma (ACC) is a rare and highly malignant tumour with a poor prognosis.
  • DIAGNOSIS: In case of an adrenal mass, hormonal workup before surgery is required for differential diagnosis, perioperative management, and for follow-up.
  • TREATMENT: Surgical resection is the therapeutic option of choice in stages 1 - 3.
  • In stage 4, the adrenolytic compound mitotane is part of the first-line treatment, but often needs to be combined with cytotoxic chemotherapy.
  • Most patients will eventually have a recurrence, so adjuvant treatment (mitotane/tumour bed radiation) has to be considered in high risk patients, even if randomized controlled trials on adjuvant treatment are still lacking.
  • STRUCTURAL PROGRESS: Several national and European structures have recently been established in order to increase our knowledge of ACC, improve therapeutic options and diagnostic procedures, and promote research.
  • GANIMED, as a Germany-wide network of experts on adrenal diseases, has been founded allowing for improved gathering of data and joint studies.
  • ENSAT (European Network for the Study of Adrenal Tumours) has been brought to life, aiming at European standards for therapy, diagnosis and tumour banking.
  • France and Italy have also developed a central registry to collect nationwide data from patients with ACC.
  • For the first time, patients with metastatic/unresectable ACC can participate in a prospective controlled randomized trial comparing two different cytotoxic chemotherapy regimes (FIRM-ACT).
  • [MeSH-major] Adrenal Cortex Neoplasms / diagnosis. Adrenal Cortex Neoplasms / surgery
  • [MeSH-minor] Adrenalectomy. Clinical Trials as Topic. Diagnosis, Differential. Humans. Mutation. Treatment Outcome

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  • (PMID = 16570232.001).
  • [ISSN] 0947-7349
  • [Journal-full-title] Experimental and clinical endocrinology & diabetes : official journal, German Society of Endocrinology [and] German Diabetes Association
  • [ISO-abbreviation] Exp. Clin. Endocrinol. Diabetes
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] Germany
  • [Number-of-references] 24
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17. Liou LS, Kay R: Adrenocortical carcinoma in children. Review and recent innovations. Urol Clin North Am; 2000 Aug;27(3):403-21
Genetic Alliance. consumer health - Adrenocortical Carcinoma.

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Adrenocortical carcinoma in children. Review and recent innovations.
  • Adrenocortical carcinoma in childhood is a rare potentially fatal disease.
  • Despite its often dramatic presentation, there typically has been a distressingly long delay between the onset of symptoms and the time of diagnosis.
  • This delay undoubtedly has contributed to the historically poor prognosis in these children by permitting the disease to reach an advanced stage before treatment is started.
  • Aggressive complete surgical resection continues to be the mainstay of treatment and is the best prognosticator of overall survival.
  • The role of adjuvant therapy and chemotherapy continues to evolve.
  • Molecular studies have increased understanding of cancer biology and may provide possible novel therapeutic approaches in the future.
  • It is hoped that increased familiarity with this unusual tumor will result in earlier detection, prompt intervention, and improved survival for children with adrenocortical carcinoma.
  • [MeSH-major] Adrenal Cortex Neoplasms / diagnosis
  • [MeSH-minor] Adrenal Glands / pathology. Adrenalectomy. Diagnosis, Differential. Humans. Prognosis. Tomography, X-Ray Computed. Virilism / etiology

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  • (PMID = 10985141.001).
  • [ISSN] 0094-0143
  • [Journal-full-title] The Urologic clinics of North America
  • [ISO-abbreviation] Urol. Clin. North Am.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] UNITED STATES
  • [Number-of-references] 125
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18. Shon Ch, Mladenovski V, Petkov R, K'tev N, Mikhailov N, Gavrailov M, Trifonov D: [Diagnosis and surgical treatment of adrenocortical carcinoma. A review of the literature and report of two cases]. Khirurgiia (Sofiia); 2000;56(2):45-9
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Diagnosis and surgical treatment of adrenocortical carcinoma. A review of the literature and report of two cases].
  • Adrenocortical carcinomas (ACC) are rare and represent only 0.05 to 0.2% of all cancers.
  • Commonly most of the cases with AC are diagnosed when the neoplastic process have spread out of the suprarenal gland (stage III or IV).
  • Computed tomography and magnetic resonance imaging are the most useful diagnostic methods of ACC but the latter is more accurate, especially in estimation of the local invasion of the tumor.
  • Surgery is the main and the most effective method for treatment of both primary and recurrent ACC, and in selective cases--of metastasis.
  • The chemotherapy with mitotane has a limited role and is indicated in cases of inoperable ACC (primary or recurrent) and/or presence of metastasis.
  • Case I: a 26-years-old female with ACC in stage II, which was diagnosed incidentally by ultrasound investigation for other consideration.
  • Problems in diagnose and surgical treatment of these cases are discussed.
  • [MeSH-major] Adrenal Cortex Neoplasms / diagnosis. Adrenal Cortex Neoplasms / surgery. Adrenocortical Carcinoma / diagnosis. Adrenocortical Carcinoma / surgery
  • [MeSH-minor] Adult. Female. Humans. Magnetic Resonance Imaging. Middle Aged. Tomography, X-Ray Computed. Treatment Outcome. Ultrasonography

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  • (PMID = 11484289.001).
  • [ISSN] 0450-2167
  • [Journal-full-title] Khirurgii︠a︡
  • [ISO-abbreviation] Khirurgiia (Sofiia)
  • [Language] bul
  • [Publication-type] Case Reports; English Abstract; Journal Article; Review
  • [Publication-country] Bulgaria
  • [Number-of-references] 41
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19. Langer P, Bartsch D, Moebius E, Rothmund M, Nies C: Adrenocortical carcinoma--our experience with 11 cases. Langenbecks Arch Surg; 2000 Oct;385(6):393-7
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Adrenocortical carcinoma--our experience with 11 cases.
  • BACKGROUND AND AIMS: Adrenocortical carcinoma (ACC) is a rare tumour with an incidence of approximately 0.5-2 cases per million per year.
  • PATIENTS/METHODS: The data of the patients with ACC were reviewed and presenting symptoms, diagnostic procedures, treatment and results of follow-up were evaluated.
  • At the time of diagnosis, five tumours were classified as stage II, two as stage III and four as stage IV.
  • Five patients received additional chemotherapy.
  • Five of the 11 patients are still alive (three stage II, one stage III and one stage IV at the time of diagnosis), three of whom have no evidence of disease (14, 48 and 71 months after surgery).
  • There is no consensus concerning adjuvant therapy.

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  • (PMID = 11127523.001).
  • [ISSN] 1435-2443
  • [Journal-full-title] Langenbeck's archives of surgery
  • [ISO-abbreviation] Langenbecks Arch Surg
  • [Language] ENG
  • [Publication-type] Journal Article
  • [Publication-country] Germany
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20. Lacroix A: Approach to the patient with adrenocortical carcinoma. J Clin Endocrinol Metab; 2010 Nov;95(11):4812-22
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Approach to the patient with adrenocortical carcinoma.
  • Adrenocortical cancer (ACC) is a rare and often aggressive malignancy that requires multidisciplinary expertise for optimal management.
  • Thorough imaging and endocrine evaluations can identify the majority of ACCs amongst adrenal tumors; however, some smaller ACCs are better identified using fluorodeoxyglucose-positron emission tomography/computed tomography scan.
  • Complete resection by an expert surgeon is the only potentially curative treatment for ACC, and tumor spillage should be avoided.
  • Despite complete resection in stage I-III tumors, approximately 40% of patients develop metastasis within 2 yr.
  • Some retrospective studies indicate that adjuvant mitotane therapy prolongs disease-free survival, leading several centers to recommend its administration; prospective studies are under way to provide future evidence-based recommendations.
  • When ACC is not surgically resectable, mitotane therapy is adjusted to reach serum levels of 14-20 μg/ml.
  • Careful replacement of glucocorticoid and mineralocorticoid deficiency after surgery or mitotane therapy is important; steroid excess from remaining tumor burden should also be controlled to avoid its morbidities.
  • For metastatic disease, combination chemotherapy should be administered, if possible, in the context of multicenter collaborative research protocols.
  • New insights in the molecular pathogenesis of ACC should allow the development of improved targeted therapies.
  • [MeSH-major] Adrenal Cortex Neoplasms / surgery. Adrenocortical Carcinoma / surgery
  • [MeSH-minor] Disease Progression. Female. Humans. Prognosis. Treatment Outcome. Young Adult

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  • (PMID = 21051577.001).
  • [ISSN] 1945-7197
  • [Journal-full-title] The Journal of clinical endocrinology and metabolism
  • [ISO-abbreviation] J. Clin. Endocrinol. Metab.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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21. Kirwan C, Carney D, O'Keefe M: Merkel cell carcinoma metastasis to the iris in a 23 year old female. Ir Med J; 2009 Feb;102(2):53-4
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Merkel cell carcinoma metastasis to the iris in a 23 year old female.
  • Merkel cell carcinoma (MCC) is a rare, aggressive neuroendocrine malignancy which predominantly affects elderly caucasians.
  • Management is stage dependant and frequently involves wide surgical excision with or without chemotherapy or radiation therapy.
  • [MeSH-major] Carcinoma, Merkel Cell / secondary. Iris / pathology. Iris Neoplasms / secondary. Skin Neoplasms / pathology
  • [MeSH-minor] Adrenal Cortex Hormones / therapeutic use. Adult. Fatal Outcome. Female. Humans. Mydriatics. Neoplasm Recurrence, Local / radiotherapy. Scalp

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  • (PMID = 19405321.001).
  • [ISSN] 0332-3102
  • [Journal-full-title] Irish medical journal
  • [ISO-abbreviation] Ir Med J
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Ireland
  • [Chemical-registry-number] 0 / Adrenal Cortex Hormones; 0 / Mydriatics
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22. Aiba M, Fujibayashi M: Histopathological diagnosis and prognostic factors in adrenocortical carcinoma. Endocr Pathol; 2005;16(1):13-22
Genetic Alliance. consumer health - Adrenocortical Carcinoma.

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Histopathological diagnosis and prognostic factors in adrenocortical carcinoma.
  • A great majority of adrenocortical tumors are benign, and many adrenocortical carcinomas (ACC) are obviously malignant at presentation.
  • The histopathological diagnosis of ACC is occasionally difficult, particularly with stage I and stage II disease.
  • Surgery is the major treatment, with chemotherapy and radiotherapy being applicable to only restricted patients.
  • The Weiss criteria are useful in diagnosing the common adult type of ACC.
  • In this article, we first describe the current histopathological diagnostic and prognostic factors of ACC, highlighting the special types of ACC to which Weiss's criteria are not fully applicable.
  • These special type tumors include pediatric adrenocortical tumors, oncocytomas, and aldosterone-producing tumors of pure zona glomerulosa type.
  • Then we present three cases with unusual small adrenocortical tumors.
  • [MeSH-major] Adrenal Cortex Neoplasms / diagnosis. Adrenocortical Carcinoma / diagnosis

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  • (PMID = 16000842.001).
  • [ISSN] 1046-3976
  • [Journal-full-title] Endocrine pathology
  • [ISO-abbreviation] Endocr. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 4964P6T9RB / Aldosterone; 67763-97-7 / Insulin-Like Growth Factor II
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23. Kendrick ML, Lloyd R, Erickson L, Farley DR, Grant CS, Thompson GB, Rowland C, Young WF Jr, van Heerden JA: Adrenocortical carcinoma: surgical progress or status quo? Arch Surg; 2001 May;136(5):543-9
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Adrenocortical carcinoma: surgical progress or status quo?
  • HYPOTHESIS: Outcome of patients with adrenocortical carcinoma (ACC) has improved with the advent of more widely available and higher quality imaging.
  • Operative management strategies and use of adjuvant therapy have not changed.
  • DESIGN: Retrospective review of patient histories, imaging studies, operative data, adjuvant therapy, and outcomes at a single institution.
  • MAIN OUTCOME MEASURES: Determinants of recurrence, survival, and the effect of adjuvant therapy on overall outcome.
  • Stage according to the TNM staging system (AJCC Cancer Staging Manual) at presentation was I (n = 0), II (n = 30), III (n = 7), and IV (n = 21).
  • Recurrence occurred in 30 patients (73%) with a median time to recurrence of 17 months.
  • Prognostic factors (P<.05) included functional status, stage, and chemotherapy in stage III/IV patients.
  • (1) Surgical resection remains the principal treatment for stage I to III disease. (2) Adjuvant therapy may improve survival in patients with stage III or IV disease. (3) Current patients were more likely to present at an earlier stage, undergo curative resections, and have improved 5-year survival than institutional historical comparisons.
  • [MeSH-major] Adrenal Cortex Neoplasms / surgery

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  • (PMID = 11343545.001).
  • [ISSN] 0004-0010
  • [Journal-full-title] Archives of surgery (Chicago, Ill. : 1960)
  • [ISO-abbreviation] Arch Surg
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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24. Hanna AM, Pham TH, Askegard-Giesmann JR, Grams JM, Iqbal CW, Stavlo P, Moir CR: Outcome of adrenocortical tumors in children. J Pediatr Surg; 2008 May;43(5):843-9
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Outcome of adrenocortical tumors in children.
  • PURPOSE: This study reviews adrenocortical tumors in children to determine factors that significantly affect outcome.
  • METHODS: An institutional review board-approved retrospective review from 1976 to 2005 identified 23 patients younger than 19 years old with histologic confirmation of adrenocortical carcinoma (ACC) and adenomas.
  • RESULTS: The mean age of the 23 children was 9.0 +/- 1.6 years; girls predominated (female-to-male ratio = 1.9:1) as did cancers (ACC 16, adenoma 7); tumor hormone production (74%); and advanced stage for disease (66%).
  • Three patients received chemotherapy or chemoradiation as primary treatment without surgery.
  • Surgical cure without adjuvant therapy was achieved for all adenomas and ACC stages I and II.
  • For ACC stage III and IV, median survival was 21 months, 5-year survival was 0%.
  • All advanced-staged ACC received adjuvant therapy.
  • The high percentage of children with functioning tumors suggests earlier detection is possible.
  • [MeSH-major] Adenoma / mortality. Adenoma / surgery. Adrenal Cortex Neoplasms / mortality. Adrenal Cortex Neoplasms / surgery. Adrenocortical Carcinoma / mortality. Adrenocortical Carcinoma / surgery
  • [MeSH-minor] Adolescent. Adrenalectomy. Chemotherapy, Adjuvant. Child. Child, Preschool. Female. Humans. Infant. Infant, Newborn. Male. Neoplasm Staging. Radiotherapy, Adjuvant. Retrospective Studies. Survival Rate. Treatment Outcome

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  • (PMID = 18485950.001).
  • [ISSN] 1531-5037
  • [Journal-full-title] Journal of pediatric surgery
  • [ISO-abbreviation] J. Pediatr. Surg.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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25. Das S, Tunuguntla HS: Balanitis xerotica obliterans--a review. World J Urol; 2000 Dec;18(6):382-7

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Many cases of BXO occurring after circumcision may be cases of secondary phimosis due to BXO not being recognized at the time of surgery.
  • Biopsy of the lesions is not essential in all cases and is indicated to differentiate from penile cancer and in atypical cases.
  • Early diagnosis and treatment of BXO are very important in preventing the urological complications of the diseases such as urethral stricture.
  • Treatment of BXO depends on the anatomic location of the lesions and their extent and severity, together with the rapidity of progression of the disease process.
  • The treatment may vary from topical corticosteroids, laser vaporization in early cases to meatoplasty and urethroplasty in extensive cases.
  • Topical pharmacotherapy is useful in the early stages to reduce the initial symptoms and slow down the progression, but is not effective in all cases and is not the curative treatment of disease.
  • Meatal stenosis, phimosis, scar adhesions, fissures, erosions of glans and prepuce and involvement of the urethra are indications for surgical treatment.
  • Surgery seems to be the only treatment that can relieve the symptoms of advanced disease.
  • BXO involving anterior urethra can be treated by 2-stage urethroplasty or substitution urethroplasty.
  • The complete excision of the stricture and flap urethroplasty seems to be better than a 2-stage procedure.
  • However, at the present time, it is not possible to say that surgery can completely resolve this chronic and progressive disease.
  • Despite many reports in the literature of cases of BXO associated with squamous cell carcinoma, the etiologic relationship between the two conditions is uncertain.

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  • (PMID = 11204255.001).
  • [ISSN] 0724-4983
  • [Journal-full-title] World journal of urology
  • [ISO-abbreviation] World J Urol
  • [Language] ENG
  • [Publication-type] Journal Article; Review
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Adrenal Cortex Hormones
  • [Number-of-references] 27
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26. Stewart JN, Flageole H, Kavan P: A surgical approach to adrenocortical tumors in children: the mainstay of treatment. J Pediatr Surg; 2004 May;39(5):759-63

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] A surgical approach to adrenocortical tumors in children: the mainstay of treatment.
  • BACKGROUND: Adrenocortical tumors (ACTs) are rare in the pediatric population.
  • Information on age, sex, presenting symptoms, hormonal levels, pathology, stage, treatment, and outcome was obtained.
  • Of the carcinomas, 3 were stage I, and 5 were stage II.
  • Two patients were treated with chemotherapy because of large tumor size and nodal involvement.
  • CONCLUSIONS: This study shows that surgical excision continues to be the mainstay of treatment for ACTs.
  • The role of adjuvant chemotherapy remains unclear because most of the children in our series were effectively treated with surgical resection only.
  • Patients should be enrolled in multicenter trials to assess the added value of chemotherapy.
  • [MeSH-major] Adrenal Cortex Neoplasms / surgery. Adrenocortical Adenoma / surgery. Adrenocortical Carcinoma / surgery
  • [MeSH-minor] Child. Child, Preschool. Combined Modality Therapy. Female. Follow-Up Studies. Humans. Infant. Lymph Nodes / pathology. Male. Neoplasm Staging

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  • (PMID = 15137014.001).
  • [ISSN] 1531-5037
  • [Journal-full-title] Journal of pediatric surgery
  • [ISO-abbreviation] J. Pediatr. Surg.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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27. Tauchmanovà L, Colao A, Marzano LA, Sparano L, Camera L, Rossi A, Palmieri G, Marzano E, Salvatore M, Pettinato G, Lombardi G, Rossi R: Andrenocortical carcinomas: twelve-year prospective experience. World J Surg; 2004 Sep;28(9):896-903

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Adrenocortical carcinoma (AC) is a rare tumor with poor prognosis.
  • Twenty-two patients (14 F, 8 M; age 22 to 59 years; median, 43 years) with AC were evaluated prospectively in a single center: tumor stage was I-II in 12 cases and III-IV in 10.
  • At diagnosis, 14 patients (63.5%) presented with features of clear adrenocortical hyperactivity.
  • The most common clinical finding was a recent diagnosis of moderate-to-severe hypertension (68%), poorly controlled by pharmacological treatment, often associated with multiple cardiovascular risk factors.
  • Response to treatments other than surgery (mitotane chemotherapy) was better in patients treated early after the first surgery.
  • In conclusion, curative surgery was the most effective treatment.
  • Monitoring arterial pressure, endocrine parameters, and metabolic parameters can be helpful for the early detection of AC recurrences.
  • [MeSH-major] Adrenal Cortex Neoplasms / surgery. Adrenocortical Carcinoma / surgery
  • [MeSH-minor] Adult. Female. Humans. Male. Middle Aged. Prospective Studies. Time Factors. Treatment Outcome

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  • (PMID = 15593464.001).
  • [ISSN] 0364-2313
  • [Journal-full-title] World journal of surgery
  • [ISO-abbreviation] World J Surg
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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28. Aspinall SR, Imisairi AH, Bliss RD, Scott-Coombes D, Harrison BJ, Lennard TW: How is adrenocortical cancer being managed in the UK? Ann R Coll Surg Engl; 2009 Sep;91(6):489-93
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] How is adrenocortical cancer being managed in the UK?
  • INTRODUCTION: Adrenocortical carcinomas are rare.
  • This case series is reported to give an overview of how adrenocortical carcinoma is currently managed in the UK.
  • PATIENTS AND METHODS: A retrospective review was made of case notes from patients with adrenocortical carcinomas presenting to the authors (TWJL, RDB, BJH, and DS-C) over the past 10 years in Newcastle, Sheffield and Cardiff.
  • Adjuvant mitotane was given in 64% of patients, in combination with cytotoxic chemotherapy in 20%.
  • One-third of patients did not receive any adjuvant therapy.
  • CONCLUSIONS: The size of tumour, stage and mode of presentation, age and overall survival of patients in this study are comparable to published series of adrenocortical carcinomas from major endocrine surgical centres world-wide.
  • Despite controversies about benefits, adjuvant mitotane was used in the majority of cases, whereas cytotoxic chemotherapy was only used in the minority.
  • The exact role of adjuvant therapy in the management of adrenocortical carcinoma is not as well established as for other more common malignancies.
  • Establishing a database for adrenocortical carcinomas in the UK would contribute to our understanding of the management of this disease.
  • [MeSH-major] Adrenal Cortex Neoplasms / drug therapy. Adrenocortical Carcinoma / drug therapy. Antineoplastic Agents, Hormonal / therapeutic use. Mitotane / therapeutic use
  • [MeSH-minor] Adrenalectomy / methods. Antineoplastic Combined Chemotherapy Protocols. Chemotherapy, Adjuvant. Cytotoxins / therapeutic use. Great Britain. Humans. Neoplasm Recurrence, Local. Retrospective Studies. Survival Analysis

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  • (PMID = 19558758.001).
  • [ISSN] 1478-7083
  • [Journal-full-title] Annals of the Royal College of Surgeons of England
  • [ISO-abbreviation] Ann R Coll Surg Engl
  • [Language] eng
  • [Publication-type] Journal Article; Multicenter Study
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antineoplastic Agents, Hormonal; 0 / Cytotoxins; 78E4J5IB5J / Mitotane
  • [Other-IDs] NLM/ PMC2966201
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29. Ciftci AO, Senocak ME, Tanyel FC, Büyükpamukçu N: Adrenocortical tumors in children. J Pediatr Surg; 2001 Apr;36(4):549-54

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Adrenocortical tumors in children.
  • BACKGROUND/PURPOSE: Etiopathogenesis and management of pediatric adrenocortical tumors (ACTs) is still obscure because of the limited number of cases.
  • Information recorded for each patient included age, sex, clinical characteristics, diagnostic methods, stage of disease, treatment, pathologic findings, and outcome.
  • The patients were subdivided into 2 groups: group I, patients with adrenocortical carcinoma (ACC) and group II, patients with adrenocortical adenoma (ACA).
  • The mean time from initial symptoms to diagnosis was 8.1 +/- 0.2 months, and this interval was similar in 2 groups, in functional and nonfunctional tumors, and in both sexes.
  • Ultrasound scan, computerized tomography, magnetic resonance imaging, intravenous pyelography, and angiography were used for the diagnosis.
  • Adjuvant chemotherapy consisting of mitotane (n = 12), mitotane plus cisplatin and etoposide (n = 2) was commenced.
  • For the time being, the most important aspect of therapy for ACCs is early diagnosis and total excision.
  • Partial excision and advanced-stage disease are the major determinants of poor outcome.
  • Because of the steadily increasing incidence of precancerous genetic syndromes of adrenal glands and poor prognosis of ACCs, childhood patients of endocrine disorders should receive a detailed and vigorous diagnostic evaluation and appropriate treatment as given to adults.
  • Patients with ACTs should be entered into multi-institutional trials to adequately assess effective chemotherapy and radiotherapy protocols and molecular mechanisms of oncogenesis.
  • [MeSH-major] Adenocarcinoma / diagnosis. Adenocarcinoma / surgery. Adenoma / diagnosis. Adenoma / surgery. Adrenal Cortex Neoplasms / diagnosis. Adrenal Cortex Neoplasms / surgery
  • [MeSH-minor] Adolescent. Adrenalectomy. Angiography. Biopsy, Needle. Child. Child, Preschool. Disease-Free Survival. Female. Follow-Up Studies. Humans. Magnetic Resonance Imaging. Male. Probability. Retrospective Studies. Statistics, Nonparametric. Survival Rate. Tomography, X-Ray Computed. Treatment Outcome

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  • [Copyright] Copyright 2001 by W.B. Saunders Company.
  • (PMID = 11283875.001).
  • [ISSN] 0022-3468
  • [Journal-full-title] Journal of pediatric surgery
  • [ISO-abbreviation] J. Pediatr. Surg.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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30. Vacca M, Krawczyk M, Petruzzelli M, Sasso RC, van Erpecum KJ, Palasciano G, van Berge-Henegouwen GP, Moschetta A, Portincasa P: Current treatments of primary sclerosing cholangitis. Curr Med Chem; 2007;14(19):2081-94
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Current treatments of primary sclerosing cholangitis.
  • End-stage liver disease and bile duct carcinoma represent frequent complications.
  • Since the aetiology of PSC remains poorly defined, medical therapy is currently limited to symptom improvement and prolonged survival.
  • Orthotopic liver transplantation (OLT) is at the moment the only definitive approach although disease relapse has been reported.
  • In this article the state of the art in PSC treatment and future promises in this field are reviewed.
  • [MeSH-major] Cholangitis, Sclerosing / drug therapy. Cholangitis, Sclerosing / surgery
  • [MeSH-minor] Adrenal Cortex Hormones / therapeutic use. Anti-Bacterial Agents / therapeutic use. Cholagogues and Choleretics / therapeutic use. Humans. Hypolipidemic Agents / therapeutic use. Immunosuppressive Agents / therapeutic use. Liver Transplantation. Ursodeoxycholic Acid / therapeutic use

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  • (PMID = 17691949.001).
  • [ISSN] 0929-8673
  • [Journal-full-title] Current medicinal chemistry
  • [ISO-abbreviation] Curr. Med. Chem.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] Netherlands
  • [Chemical-registry-number] 0 / Adrenal Cortex Hormones; 0 / Anti-Bacterial Agents; 0 / Cholagogues and Choleretics; 0 / Hypolipidemic Agents; 0 / Immunosuppressive Agents; 724L30Y2QR / Ursodeoxycholic Acid
  • [Number-of-references] 219
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31. Sezaki H, Suzuki F, Hosaka T, Akuta N, Someya T, Kobayashi M, Suzuki Y, Saitoh S, Arase Y, Ikeda K, Kumada H: Long-term follow-up of HBeAg-positive young adult Japanese patients treated with corticosteroid withdrawal therapy for chronic hepatitis B. Intervirology; 2006;49(6):339-45
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Long-term follow-up of HBeAg-positive young adult Japanese patients treated with corticosteroid withdrawal therapy for chronic hepatitis B.
  • OBJECTIVES: To evaluate the long-term effects of corticosteroid withdrawal therapy (CSWT) in young adult Japanese patients with chronic hepatitis B (CH-B) virus infection.
  • RESULTS: Retreatment was not required in 41 patients (38.7%; retreatment(-) group) while 65 (61.3%) received treatment after the initial CSWT (retreatment(+) group).
  • Larger proportions of patients of the retreatment(-) group were females, had liver histology stage F2/F3, high ICG R15, and genotypes A/B/D/E, compared with the retreatment(+) group.
  • In the retreatment(-) group, the rate of liver cirrhosis (LC; 7.3%, 3 patients) was lower, but the rate of hepatocellular carcinoma (HCC; 12.2%, 5 patients) was higher than in the retreatment(+) group (20%, 13 patients, and 4.6%, 3 patients, respectively).
  • CONCLUSIONS: Our results suggest that CSWT is good short-term therapy and has possible long-term effects in young adult Japanese patients with CH-B.
  • [MeSH-major] Adrenal Cortex Hormones / therapeutic use. Hepatitis B e Antigens / blood. Hepatitis B, Chronic / drug therapy
  • [MeSH-minor] Adolescent. Adult. Carcinoma, Hepatocellular / etiology. Child. Drug Administration Schedule. Female. Follow-Up Studies. Humans. Japan. Liver / pathology. Liver Cirrhosis / etiology. Liver Neoplasms / etiology. Male. Prognosis

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  • [Copyright] Copyright (c) 2006 S. Karger AG, Basel.
  • (PMID = 16926546.001).
  • [ISSN] 0300-5526
  • [Journal-full-title] Intervirology
  • [ISO-abbreviation] Intervirology
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Switzerland
  • [Chemical-registry-number] 0 / Adrenal Cortex Hormones; 0 / Hepatitis B e Antigens
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32. Czaja AJ: Current and future treatments of autoimmune hepatitis. Expert Rev Gastroenterol Hepatol; 2009 Jun;3(3):269-91
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Current and future treatments of autoimmune hepatitis.
  • Corticosteroid therapy induces clinical, laboratory and histological improvements in 80% of patients with autoimmune hepatitis.
  • The combination regimen is preferred and treatment should be considered in all patients with active disease.
  • The duration of therapy is finite and the medication should be discontinued after resolution of all manifestations of inflammatory activity, including the histological changes.
  • Relapse after drug withdrawal occurs in 50-79% of patients, and it should be treated with long-term azathioprine (2 mg/kg daily).
  • Salvage therapies for individuals intolerant of or refractory to the conventional regimens include high-dose corticosteroids, with or without high-dose azathioprine, 6-mercaptopurine, mycophenolate mofetil, tacrolimus or ciclosporin.
  • Liver transplantation should be considered in patients with hepatic failure unresponsive to corticosteroid treatment, decompensated cirrhosis with a Model for End-Stage Liver Disease score of at least 15 points, or hepatocellular carcinoma that meets transplantation criteria.
  • Future therapies are likely to include mesenchymal stem cell transplantation, adoptive transfer of T regulatory cells, and cytokine manipulation.
  • The emergence of new treatments will require the development of a collaborative network of clinical and basic investigators, as the complexity and specificity of current management problems require solutions that exceed the capabilities of single institutions.
  • [MeSH-major] Adrenal Cortex Hormones / therapeutic use. Hepatitis, Autoimmune / drug therapy. Immunosuppressive Agents / therapeutic use
  • [MeSH-minor] Azathioprine / therapeutic use. Dose-Response Relationship, Drug. Drug Therapy, Combination. Humans. Prednisone / therapeutic use

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  • (PMID = 19485809.001).
  • [ISSN] 1747-4132
  • [Journal-full-title] Expert review of gastroenterology & hepatology
  • [ISO-abbreviation] Expert Rev Gastroenterol Hepatol
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Adrenal Cortex Hormones; 0 / Immunosuppressive Agents; MRK240IY2L / Azathioprine; VB0R961HZT / Prednisone
  • [Number-of-references] 242
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