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1. Almogy G, Lieberman S, Gips M, Pappo O, Edden Y, Jurim O, Simon Slasky B, Uzieli B, Eid A: Clinical outcomes of surgical resections for primary liver sarcoma in adults: results from a single centre. Eur J Surg Oncol; 2004 May;30(4):421-7
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • BACKGROUND: Primary hepatic sarcoma is a rare tumour with a poor prognosis.
  • The clinical characteristics, imaging findings, surgical procedures, adjuvant therapy and outcome were retrospectively reviewed.
  • There were two patients each with angiosarcoma (AS), leiomyosarcoma (LMS), and undifferentiated embryonal sarcoma (UES), one patient with epithelioid hemangioendothelioma (EHE) and one patient with malignant peripheral nerve sheath sarcoma (PNSS).
  • Preoperative diagnosis of a primary liver sarcoma was made in 7/8 cases, either by fine needle aspiration (n = 5) or angiography (n = 2).
  • Two patients developed complications and there was one death.
  • Systemic chemotherapy led to tumour regression in both patients with UES which enabled a second hepatic resection.
  • Differentiating the rare primary liver sarcoma from the much more common hepatocellular carcinoma (HCC) may aid in planning therapy.
  • Liver resection combined with adjuvant chemotherapy are the mainstays of treatment for UES in the adult.
  • [MeSH-minor] Adult. Aged. Chemotherapy, Adjuvant. Diagnosis, Differential. Female. Hepatectomy / methods. Humans. Length of Stay. Liver Function Tests. Male. Middle Aged. Retrospective Studies. Tomography, X-Ray Computed. Treatment Outcome

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  • (PMID = 15063896.001).
  • [ISSN] 0748-7983
  • [Journal-full-title] European journal of surgical oncology : the journal of the European Society of Surgical Oncology and the British Association of Surgical Oncology
  • [ISO-abbreviation] Eur J Surg Oncol
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
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2. Kinkor Z, Hes O: [Pleomorphic epithelioid/clear cell malignant tumor of the uterus exhibiting both myoid and melanocytic differentiation--leiomyosarcoma or PEComa? A case report and a review of the literature]. Cesk Patol; 2007 Jul;43(3):103-8
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Pleomorphic epithelioid/clear cell malignant tumor of the uterus exhibiting both myoid and melanocytic differentiation--leiomyosarcoma or PEComa? A case report and a review of the literature].
  • [Transliterated title] Pleomorfni epiteloidni a svetlobunecný maligní tumor delohy s myoidní a melanocytární diferenciací--leiomyosarkom nebo PECom? Kazuistika a prehled literatury.
  • Microscopically, the tumor displayed solid mosaic pattern and consisted of large epithelioid cells with ample eosinophilic, finely granular cytoplasm ongoing apparent clear cell change elsewhere.
  • No obvious stigmata of tuberous sclerosis were found and a five months follow-up after chemotherapy indicated no progression of disease.
  • With some uncertainty the tumor was finally rendered as pleomorphic leiomyosarcoma with peculiar melanocytic differentiation.
  • Reviewed is the literature and discussed is the differential diagnosis.
  • [MeSH-major] Leiomyosarcoma / pathology. Uterine Neoplasms / pathology
  • [MeSH-minor] Adult. Epithelioid Cells / pathology. Female. Humans


3. Toyoshima M, Okamura C, Niikura H, Ito K, Yaegashi N: Epithelioid leiomyosarcoma of the uterine cervix: a case report and review of the literature. Gynecol Oncol; 2005 Jun;97(3):957-60
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Epithelioid leiomyosarcoma of the uterine cervix: a case report and review of the literature.
  • BACKGROUND: Epithelioid leiomyosarcomas arising from the uterine cervix are extremely rare neoplasms, with only three cases reported in the English literature.
  • Histological findings, including immunohistochemical study using desmin, SMA, cytokeratin, S-100, HMB-45, vimentin, melan-A, and CD68, led to a diagnosis of epithelioid leiomyosarcoma of the uterine cervix.
  • The patient underwent adjuvant chemotherapy and has been disease-free for over 20 months.
  • CONCLUSION: Immunohistochemical studies may be needed to differentiate among the alternative diagnoses of malignant melanoma, metastatic carcinoma, and epithelioid sarcoma.
  • [MeSH-major] Leiomyosarcoma / pathology. Uterine Cervical Neoplasms / pathology


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4. Kato K, Arai K, Tanaka Y, Ijiri R, Kato Y, Kigasawa H, Toyoda Y, Aida N, Ohama Y: Epithelioid leiomyosarcoma in a non-immunocompromised infant: additional differential diagnosis of pediatric "round cell tumors". Mod Pathol; 2000 Oct;13(10):1156-60
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  • [Title] Epithelioid leiomyosarcoma in a non-immunocompromised infant: additional differential diagnosis of pediatric "round cell tumors".
  • We report an 18-month-old Japanese girl with purely epithelioid leiomyosarcoma presenting as a huge intraabdominal mass.
  • Silver stain, however, demonstrated well-developed reticulin fibers often outlining individual tumor cells.
  • The diagnosis of epithelioid leiomyosarcoma was made.
  • Following reduction in tumor size by chemotherapy, the serum NSE level was normalized.
  • Although extremely rare, epithelioid leiomyosarcoma should be added in the list of differential diagnoses of pediatric "round cell tumors. "
  • [MeSH-major] Abdominal Neoplasms / pathology. Immunocompromised Host. Leiomyosarcoma / pathology. Soft Tissue Neoplasms / pathology
  • [MeSH-minor] Chemotherapy, Adjuvant. Diagnosis, Differential. Female. Humans. Infant. Magnetic Resonance Imaging

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  • (PMID = 11048812.001).
  • [ISSN] 0893-3952
  • [Journal-full-title] Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc
  • [ISO-abbreviation] Mod. Pathol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] UNITED STATES
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5. Silva EG, Deavers MT, Bodurka DC, Malpica A: Uterine epithelioid leiomyosarcomas with clear cells: reactivity with HMB-45 and the concept of PEComa. Am J Surg Pathol; 2004 Feb;28(2):244-9
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Uterine epithelioid leiomyosarcomas with clear cells: reactivity with HMB-45 and the concept of PEComa.
  • In this study, we investigated HMB-45 expression in epithelioid uterine leiomyosarcomas with clear cell areas.
  • From 12 epithelioid leiomyosarcomas, we selected 5 that had:.
  • Two received radiotherapy, and three were also treated with chemotherapy.
  • The epithelioid component varied from 50% to 90% in each case; and the percentage of clear cells was < 1% in one case, 5% in one case, and 10% to 80% in three cases.
  • Unequivocal uterine epithelioid leiomyosarcomas may have clear cells positive for HMB-45.
  • These tumors might belong to the group of lesion designated as PEComas; however, it is advisable to designate them as uterine leiomyosarcomas.
  • In uterine smooth muscle tumors, some epithelioid cells most likely undergo clear cell changes and become positive for HMB-45.
  • It would be advisable to perform this stain in all epithelioid smooth muscle tumors of the uterus.
  • [MeSH-major] Leiomyosarcoma / metabolism. Leiomyosarcoma / pathology. Neoplasm Proteins / metabolism. Uterine Neoplasms / metabolism. Uterine Neoplasms / pathology

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  • (PMID = 15043315.001).
  • [ISSN] 0147-5185
  • [Journal-full-title] The American journal of surgical pathology
  • [ISO-abbreviation] Am. J. Surg. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antigens, Neoplasm; 0 / Melanoma-Specific Antigens; 0 / Neoplasm Proteins
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6. Gülicher D, Aumann V, Hauptmann K, Gerlach KL: [Epitheloid leiomyosarcoma of the mouth in childhood]. Klin Padiatr; 2005 Sep-Oct;217(5):291-6
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  • [Title] [Epitheloid leiomyosarcoma of the mouth in childhood].
  • [Transliterated title] Epitheloides Leiomyosarkom der Mundhöhle.
  • Histologic and immunohistochemical examination revealed a poorly differentiated epitheloid leiomyosarcoma with destruction of the mandible.
  • According to the CWS-96-study the patient underwent preoperative chemotherapy followed by complete resection of the mandibular body with the surrounding soft tissues.
  • The further treatment consisted of tumor resection, oral chemotherapy and irradiation.
  • The patient died of progressive disease 16 months after diagnosis.
  • Leiomyosarcoma is extremely uncommon in childhood, especially with localisation in the oral cavity.
  • Diagnosis is based on histologic examination and immunohistochemistry.
  • In the presented case the lack of smooth muscle actin expression made diagnosis difficult.
  • The preoperative chemotherapy could not achieve reduction of tumor size, so that extensive surgery became necessary.
  • Estimation of the prognosis of the oral leiomyosarcomas in childhood is difficult.
  • [MeSH-major] Leiomyosarcoma. Mouth Neoplasms
  • [MeSH-minor] Adolescent. Adult. Antineoplastic Agents / therapeutic use. Biopsy. Child. Child, Preschool. Combined Modality Therapy. Female. Humans. Immunohistochemistry. Infant. Mouth Floor / pathology. Neoplasm Recurrence, Local. Preoperative Care. Prognosis. Time Factors. Treatment Outcome

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  • (PMID = 16167278.001).
  • [ISSN] 0300-8630
  • [Journal-full-title] Klinische Pädiatrie
  • [ISO-abbreviation] Klin Padiatr
  • [Language] ger
  • [Publication-type] Case Reports; Comparative Study; English Abstract; Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Antineoplastic Agents
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7. Fleshman R, Mayerson J, Wakely PE Jr: Fine-needle aspiration biopsy of high-grade sarcoma: a report of 107 cases. Cancer; 2007 Dec 25;111(6):491-8
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • They also searched their tissue database for all HGS cases that had prior FNA biopsy findings.
  • RESULTS: A total of 107 FNA samples from 98 patients (age range, 13-90 years, with a male:female ratio of 1:1) had an FNA diagnosis of HGS, or had HGS and a prior FNA diagnosis of another entity.
  • The positive predictive value of an FNA diagnosis of HGS was 97% (88 of 91 cases).
  • Fifty-four cases were diagnosed as HGS, not otherwise specified, 8 as myxofibrosarcoma, 8 as osteosarcoma, 5 as malignant peripheral nerve sheath tumor, 5 as leiomyosarcoma, 4 as Ewing sarcoma, 4 as liposarcoma, 2 as epithelioid sarcoma, and 1 as angiosarcoma.
  • Approximately 71% of patients presented with a primary tumor, 23% with disease recurrence, and 7% with metastasis.
  • FNA diagnosis was confirmed histologically in 88% of cases, clinically in 7% of cases, and cytogenetically in 1% of cases; 3% of cases had false-positive results and 1 patient was lost to follow-up.
  • Sixteen of 19 patients received neoadjuvant chemotherapy based on the FNA diagnosis alone.
  • CONCLUSIONS: A cytopathologic diagnosis of HGS was found to be accurate in 88 of 97 cases (91%) with follow-up.
  • A FNA biopsy diagnosis of HGS appears to be clinically reliable in a high percentage of cases when used in close conjunction with the orthopedic team.
  • [MeSH-major] Biopsy, Fine-Needle. Cytodiagnosis. Sarcoma / diagnosis. Soft Tissue Neoplasms / diagnosis
  • [MeSH-minor] Adolescent. Adult. Aged. Aged, 80 and over. Antineoplastic Agents / therapeutic use. Bone Neoplasms / diagnosis. Female. Humans. Male. Middle Aged. Neoadjuvant Therapy. Predictive Value of Tests. Reproducibility of Results

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  • (PMID = 17941014.001).
  • [ISSN] 0008-543X
  • [Journal-full-title] Cancer
  • [ISO-abbreviation] Cancer
  • [Language] eng
  • [Publication-type] Evaluation Studies; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents
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8. Ryan P, Nguyen VH, Gholoum S, Carpineta L, Abish S, Ahmed NN, Laberge JM, Riddell RH: Polypoid PEComa in the rectum of a 15-year-old girl: case report and review of PEComa in the gastrointestinal tract. Am J Surg Pathol; 2009 Mar;33(3):475-82
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • PEComa of the gastrointestinal tract, composed of perivascular epithelioid cells with myomelanocytic differentiation, is rare with previous literature limited to 16 case reports.
  • We report PEComa with lymph node involvement occurring in the rectum of a 15-year-old girl, treated by surgical resection and adjuvant chemotherapy.
  • We review the differential diagnosis of intestinal PEComa, which includes malignant melanoma, epithelioid gastrointestinal stromal tumors, clear cell sarcoma of soft parts, alveolar soft part sarcoma, leiomyosarcoma with HMB45 expression, and paraganglioma.
  • [MeSH-major] Perivascular Epithelioid Cell Neoplasms / pathology. Rectal Neoplasms / pathology
  • [MeSH-minor] Adolescent. Antineoplastic Agents / therapeutic use. Combined Modality Therapy. Diagnosis, Differential. Digestive System Surgical Procedures. Female. Humans. Immunohistochemistry. Microscopy, Electron, Transmission. Reverse Transcriptase Polymerase Chain Reaction. Tomography, X-Ray Computed

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  • (PMID = 19092636.001).
  • [ISSN] 1532-0979
  • [Journal-full-title] The American journal of surgical pathology
  • [ISO-abbreviation] Am. J. Surg. Pathol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents
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9. Poggio JL, Nagorney DM, Nascimento AG, Rowland C, Kay P, Young RM, Donohue JH: Surgical treatment of adult primary hepatic sarcoma. Br J Surg; 2000 Nov;87(11):1500-5
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Surgical treatment of adult primary hepatic sarcoma.
  • Optimal therapeutic approaches remain unclear.
  • METHODS: Twenty consecutive adult patients who had surgical treatment for primary hepatic sarcomas were reviewed.
  • Other than one patient with primary hepatic angiosarcoma who had a history of thorium dioxide colloid (Thorotrast) exposure 23 years before diagnosis, no predisposing causes were apparent.
  • No patient received neoadjuvant chemotherapy or radiotherapy but radiotherapy was delivered intraoperatively in one patient.
  • RESULTS: Leiomyosarcoma was the most common histological type of sarcoma diagnosed (five of 20 patients), followed by malignant solitary fibrous tumour (four) and epithelioid haemangioendothelioma (three).
  • Thirteen patients developed a recurrence.
  • Distant metastases (ten patients) and intrahepatic recurrence (six) were the predominant sites of initial treatment failure.
  • Six patients received salvage chemotherapy.
  • CONCLUSION: Surgical resection is the only effective therapy for primary hepatic sarcomas at present.
  • Better adjuvant therapy is necessary, especially for high-grade malignancies, owing to the high failure rate with operation alone.
  • [MeSH-minor] Adult. Aged. Aged, 80 and over. Antineoplastic Agents / therapeutic use. Disease-Free Survival. Female. Humans. Male. Middle Aged. Neoplasm Recurrence, Local / pathology. Preoperative Care / methods. Retrospective Studies

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  • (PMID = 11091236.001).
  • [ISSN] 0007-1323
  • [Journal-full-title] The British journal of surgery
  • [ISO-abbreviation] Br J Surg
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] ENGLAND
  • [Chemical-registry-number] 0 / Antineoplastic Agents
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10. Büchner-Steudel P, Fleig WE: [Diagnosis and conservative treatment of gastrointestinal stromal tumors (GIST)]. Dtsch Med Wochenschr; 2004 Aug 20;129(34-35):1808-10
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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 conservative treatment of gastrointestinal stromal tumors (GIST)].
  • [Transliterated title] Diagnostik und konservative Therapie gastrointestinaler Stromatumoren.
  • [MeSH-major] Antineoplastic Agents / therapeutic use. Enzyme Inhibitors / therapeutic use. Gastrointestinal Neoplasms / diagnosis. Gastrointestinal Neoplasms / drug therapy. Piperazines / therapeutic use. Pyrimidines / therapeutic use
  • [MeSH-minor] Benzamides. Diagnosis, Differential. Endoscopy, Gastrointestinal. Endosonography. Female. Humans. Imatinib Mesylate. Leiomyoma, Epithelioid / diagnosis. Leiomyoma, Epithelioid / drug therapy. Leiomyoma, Epithelioid / epidemiology. Leiomyosarcoma / diagnosis. Leiomyosarcoma / drug therapy. Leiomyosarcoma / epidemiology. Male. Mesenchymoma / diagnosis. Mesenchymoma / drug therapy. Mesenchymoma / epidemiology. Neurilemmoma / diagnosis. Neurilemmoma / drug therapy. Neurilemmoma / epidemiology. Prognosis. Protein-Tyrosine Kinases / antagonists & inhibitors. Sex Factors. Tomography, X-Ray Computed

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  • (PMID = 15314745.001).
  • [ISSN] 0012-0472
  • [Journal-full-title] Deutsche medizinische Wochenschrift (1946)
  • [ISO-abbreviation] Dtsch. Med. Wochenschr.
  • [Language] ger
  • [Publication-type] Journal Article; Review
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Benzamides; 0 / Enzyme Inhibitors; 0 / Piperazines; 0 / Pyrimidines; 8A1O1M485B / Imatinib Mesylate; EC 2.7.10.1 / Protein-Tyrosine Kinases
  • [Number-of-references] 16
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11. Silva EG, Bodurka DC, Scouros MA, Ayala A: A uterine leiomyosarcoma that became positive for HMB45 in the metastasis. Ann Diagn Pathol; 2005 Feb;9(1):43-5
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] A uterine leiomyosarcoma that became positive for HMB45 in the metastasis.
  • Uterine smooth muscle tumors are usually spindle cell lesions, but a minority is composed of epithelioid cells.
  • Recently, it has been shown that some of these tumors can be positive for HMB45, and some authors have advocated calling these lesions perivascular epithelioid cell (PEC) tumors or PEComas.
  • The patient involved is a 29-year-old black woman who was found to have an epithelioid leiomyosarcoma of the uterus in November 1995.
  • She was treated with a simple hysterectomy and bilateral salpingo-oophorectomy.
  • She, therefore, also received chemotherapy and was free of disease until October 2002, when a recurrent tumor was detected in her spine.
  • After undergoing resection of the lesion at 2 different times, in 2002 and 2003, the patient was treated with radiotherapy and is currently receiving chemotherapy.
  • On microscopic examination, the tumor in the uterus and liver both proved to be an epithelioid leiomyosarcomas that was diffusely positive for smooth muscle actin.
  • However, although the tumors resected from the spine in 2002 and 2003 showed features similar to those of the uterine neoplasm, but with a lower percentage of cells positive for smooth muscle actin and more clear cells, several of the clear cells were positive for HMB45.
  • [MeSH-major] Leiomyosarcoma / secondary. Liver Neoplasms / secondary. Neoplasm Proteins. Uterine Neoplasms / pathology
  • [MeSH-minor] Actins / analysis. Adult. Antigens, Neoplasm. Biomarkers, Tumor / analysis. Cell Count. Combined Modality Therapy. Epithelioid Cells / chemistry. Epithelioid Cells / pathology. Female. Humans. Melanoma-Specific Antigens. Neoplasm Metastasis / pathology. Neoplasm Metastasis / therapy

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  • (PMID = 15692950.001).
  • [ISSN] 1092-9134
  • [Journal-full-title] Annals of diagnostic pathology
  • [ISO-abbreviation] Ann Diagn Pathol
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Actins; 0 / Antigens, Neoplasm; 0 / Biomarkers, Tumor; 0 / Melanoma-Specific Antigens; 0 / Neoplasm Proteins
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12. Danikas D, Theodorou SJ, Singh R, Camal DE: Leiomyosarcoma of the gallbladder: a case report. Am Surg; 2001 Sep;67(9):873-4
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Leiomyosarcoma of the gallbladder: a case report.
  • The diagnosis is rarely made preoperatively.
  • The patient was a 51-year-old woman with a 2-month history of right upper quadrant pain, nausea, vomiting, and a 10-pound weight loss.
  • Laparoscopic cholecystectomy was converted to open as a result of dense tissue in the middle to distal gallbladder.
  • Pathology revealed poorly differentiated epithelioid leiomyosarcoma of the gallbladder with extension to the liver.
  • The disease followed a very aggressive course and the patient died 3 weeks after the procedure.
  • Recommended treatment is extensive surgical resection that can be followed by radiotherapy or chemotherapy.
  • [MeSH-major] Gallbladder Neoplasms / diagnosis. Leiomyosarcoma / diagnosis
  • [MeSH-minor] Diagnosis, Differential. Female. Gallbladder / pathology. Humans. Liver / pathology. Middle Aged

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  • (PMID = 11565767.001).
  • [ISSN] 0003-1348
  • [Journal-full-title] The American surgeon
  • [ISO-abbreviation] Am Surg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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13. Khabir A, Boudawara T, Ayadi L, Kharrat M, Kharrat M, Beyrouti I, Jlidi R: [Epithelioid bilateral ovarian leiomyosarcoma: a study]. Ann Pathol; 2003 Feb;23(1):47-9
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Epithelioid bilateral ovarian leiomyosarcoma: a study].
  • [Transliterated title] Léiomyosarcome ovarien bilatéral de type épithélioïde: une observation.
  • Primary ovarian leiomyosarcoma is extremely rare and generally affects post menopausal women.
  • Its histogenesis is not clear its prognosis seems to be improved by radical surgery and adjuvant therapy.
  • A diagnosis of bilateral ovarian epithelioid leiomyosarcoma was made on pathological examination with immunohistochemistry.
  • Adjuvant chemotherapy was given.
  • [MeSH-major] Leiomyosarcoma / diagnosis. Ovarian Neoplasms / diagnosis
  • [MeSH-minor] Biomarkers, Tumor / analysis. Chemotherapy, Adjuvant. Fallopian Tubes / surgery. Female. Humans. Hysterectomy. Immunohistochemistry. Liver Neoplasms / secondary. Liver Neoplasms / ultrasonography. Menopause. Middle Aged. Ovariectomy. Prognosis. Tomography, X-Ray Computed

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  • (PMID = 12743499.001).
  • [ISSN] 0242-6498
  • [Journal-full-title] Annales de pathologie
  • [ISO-abbreviation] Ann Pathol
  • [Language] fre
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] France
  • [Chemical-registry-number] 0 / Biomarkers, Tumor
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