Pathology international
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Pathology international · Oct 2003
Review Case ReportsRadiation-induced laryngeal angiosarcoma after cervical tuberculosis and squamous cell carcinoma: case report and review of the literature.
Primary laryngeal angiosarcoma (LA) is quite rare with only 13 cases reported in English literature to date. A case of LA after radiation therapy for tuberculosis and squamous cell carcinoma is reported. A 70-year-old woman had a history of radiation therapy for left cervical tuberculosis at the age of 28. ⋯ The patient was still alive despite multiple skin and soft tissue metastasis 3 years and 6 months after the radical operation. Distinction of postirradiation angiosarcoma from pseudoangiosarcomatous carcinoma seems difficult but is important because irradiation is not effective and an initial radical surgery is the only effective treatment. Although irradiation is a common treatment for laryngeal squamous cell carcinoma, this is only the second case of radiation-induced LA in English literature.
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Richter syndrome (RS) is well known as a secondary high-grade lymphoma, mostly diffuse large B-cell lymphoma (DLBCL) developed in patients with B-cell chronic lymphocytic leukemia (B-CLL). In this review, we describe clinicopathological, histological, immunophenotypical and genetic findings of RS. The patients with RS, regardless of transformation of pre-existing clone or de novo malignant clone, were resistant to conventional combined chemotherapy and died within months of diagnosis. ⋯ In addition, RS does not include chromosomal translocation between Ig locus and oncogenes or rearrangements of bcl-6 gene, both of which were found in some de novo DLBCL. Several candidates, such as mutation of p53 gene and abnormalities of cyclin dependent kinase inhibitor, have been proposed to play an important role in the transformation of a part of B-CLL. However, there is still uncertainty as to how B-CLL progresses or develops into RS.
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Pathology international · Apr 2003
Review Case ReportsOsteoma of the frontal sinus complicated by intracranial mucocele.
We present a rare case of intracranial mucocele associated with frontal sinus osteoma in a patient suffering from generalized convulsion. The intracranial mucocele occurs as a complication of obstruction of sinus drainage caused by osteoma, but it is often diagnosed preoperatively as an intracranial or intracerebral cyst because of the rarity of these combined lesions in neurosurgical practice. ⋯ Moreover, the differentiation of the mucocele from the intracranial endodermal cyst predominantly depends upon its continuity with the intracranial osteoma portion or the sinus. Thus, knowledge of this rare lesion is important for accurate diagnosis and clinical management.
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Pathology international · May 2002
Case ReportsPeritoneal endometriosis in the broad ligament presenting as a large tumor.
Peritoneal endometriosis presenting as a tumor is very rare. A case of peritoneal endometriosis in the broad ligament presenting as a large tumor is reported. A 39-year-old woman had a solid and cystic tumor with many microcysts, measuring 17 x 13 x 3.5 cm, mainly located in the right posterior broad ligament. ⋯ Among the six cases (five previously reported cases and our case) of tumor-like endometriosis in the peritoneum, two cases (33%) had received tamoxifen therapy and four cases (67%) had cystic or solid and cystic tumor. Among them, the tumor size ranged from 3 to 17 cm in diameter, and our case was the largest. In conclusion, this case is rare, but it is important for pathologists to be aware of the phenomenon (that extensive peritoneal endometriosis produces a large tumor) in the histological diagnosis of a tumor in the peritoneum.
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Pathology international · Aug 2001
ReviewPathogenesis of multifocal micronodular pneumocyte hyperplasia and lymphangioleiomyomatosis in tuberous sclerosis and association with tuberous sclerosis genes TSC1 and TSC2.
Tuberous sclerosis (TSC) is a rare, genetically determined disorder / familial tumor syndrome, currently diagnosed using specific clinical criteria proposed by Gomez, including the presence of multiorgan hamartomas. Pulmonary involvement in TSC is well known as pulmonary lymphangioleiomyomatosis (LAM), which has an incidence of 1-2.3% in TSC patients. LAM has immunohistochemical expression of both smooth-muscle actin and a monoclonal antibody specific for human melanoma, HMB-45. ⋯ TSC genes are considered to be tumor suppressor genes, and mutations in them may lead to abnormal differentiation and proliferation of cells. Tuberin, the TSC2 gene product, has recently been found to be expressed in LAM and MMPH. In this article we discuss the histogenesis and genetic abnormalities of neoplastic lesions associated with TSC, and we review the current understanding of the pathogenesis of pulmonary hamartomatous lesions such as LAM and MMPH in TSC.