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Titolo:
Abdominopelvic sarcoma of perivascular epithelioid cells. Report of four cases in young women, one with tuberous sclerosis
Autore:
Bonetti, F; Martignoni, G; Colato, C; Manfrin, E; Gambacorta, M; Faleri, M; Bacchi, C; Sin, VC; Wong, NL; Coady, M; Kwok-cheung, JC;
Indirizzi:
Prince Wales Hosp, Dept Anat Pathol, Sidney, BC, Canada Prince Wales HospSidney BC Canada Dept Anat Pathol, Sidney, BC, Canada Queen Elizabeth Hosp, Dept Pathol & Clin Oncol, Hong Kong, Hong Kong, Peoples R China Queen Elizabeth Hosp Hong Kong Hong Kong Peoples R China Peoples R China UNESP, Fac Med, Dept Pathol, Botucatu, SP, Brazil UNESP Botucatu SP Brazil ESP, Fac Med, Dept Pathol, Botucatu, SP, Brazil Osped Maggiore Niguarda, Serv Anat Patol, Milan, Italy Osped Maggiore Niguarda Milan Italy arda, Serv Anat Patol, Milan, Italy Univ Verona, Ist Anat Patol, I-37100 Verona, Italy Univ Verona Verona Italy I-37100 , Ist Anat Patol, I-37100 Verona, Italy
Titolo Testata:
MODERN PATHOLOGY
fascicolo: 6, volume: 14, anno: 2001,
pagine: 563 - 568
SICI:
0893-3952(200106)14:6<563:ASOPEC>2.0.ZU;2-G
Fonte:
ISI
Lingua:
ENG
Soggetto:
SUGAR TUMOR; RENAL ANGIOMYOLIPOMA; KIDNEY; LYMPHANGIOLEIOMYOMATOSIS; CARCINOMA; LESIONS; FAMILY; PATIENT; MEMBER; UTERUS;
Keywords:
perivascular epithelioid cell; angiomyolipoma; clear cell "sugar" tumor; melanoma markers; tuberous sclerosis;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Life Sciences
Citazioni:
30
Recensione:
Indirizzi per estratti:
Indirizzo: Bonetti, F Policlin Borgo Roma, Ist Anat Patol, I-37134 Verona, Italy Policlin Borgo Roma Verona Italy I-37134 -37134 Verona, Italy
Citazione:
F. Bonetti et al., "Abdominopelvic sarcoma of perivascular epithelioid cells. Report of four cases in young women, one with tuberous sclerosis", MOD PATHOL, 14(6), 2001, pp. 563-568

Abstract

The perivascular epithelioid cell has been proposed to be the unifying proliferating cell type in a number of lesions such as angiomyolipoma, lymphangiomyomatosis, clear cell "sugar" tumor and renal capsuloma. With the exception of rare examples of angiomyolipoma, they are non-metastasizing. We report four examples of a new member of this family of perivascular epithelioid cell neoplasms that occur in abdominopelvic location and show metastatic properties. The patients, all women, were aged 19 to 41 years (mean, 32), and presented with a tumor mass involving the serosa of the ileum, uterus orpelvic cavity. Morphologically, the tumors were composed of sheets of large polygonal cells with glycogen-rich clear or eosinophilic cytoplasm and moderately pleomorphic nuclei, traversed by a delicate vasculature, mimickingclear cell carcinoma. There were areas of coagulative necrosis and occasional mitotic figures. Intracytoplasmic brown pigment was present in two cases. Spindly cells, smooth muscle and fat were absent. Lymphovascular invasion was present in all, lymph node metastasis was documented in two and metastasis to the ovary was present in one case. Two patients developed widespread metastatic disease after 10 and 28 months from diagnosis. One patient showed the clinical signs of tuberous sclerosis. In spite of the epithelial-like appearance, the tumor cells were negative for epithelial markers but were strongly positive with the melanogenesis-related marker HMB45. Another melanogenesis marker (MART-1) was positive in two cases. Other markers including S-100 protein, vimentin, muscle-specific actin, desmin and chromogranin A were negative. Thus, these tumors are not readily classifiable in the existing schema of known entities, and show over-lapping morpho-phenotypic features of clear cell "sugar" tumor of the lung and epithelioid angiomyolipoma. We consider them as sarcomas composed of a pure population of uncommitted perivascular epithelioid cell, that lack modulation toward smooth muscle or adipose cells.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 18/09/20 alle ore 17:02:24