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Perivascular epithelioid cell tumors (PEComas) are rare and are characterized by the expression of myomelanocytic markers. They are a complex family that includes angiomyolipomas, lymphangioleiomyomatoses and other soft tissue and visceral tumors. Due to the low prevalence of these tumors, the natural history is unclear; furthermore, a molecular classification integrating clinical, pathological and molecular parameters has not been described to date.
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| Measure | Description | Time Frame |
|---|---|---|
| To retrospectively describe the percentage of tumors with FISH (Fluorescence In Situ Hybridization) identification of the TFE3 rearrangement out of 100 cases of PEComas diagnosed in France between 01 January 1990 and 15 September 2019 | Files analysed retrospectively from January 01, 1990 to September 15, 2019 will be examined |
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Inclusion criteria:
Exclusion criteria :
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patients having PEComas diagnosed in France between 01/01/1990 and 15/09/2019 and reviewed by a sarcoma referral pathologist
| Name | Role | Phone | Extension | |
|---|---|---|---|---|
| Gabriel MALOUF, MD, PhD | Contact | 33 3 88 11 51 41 | malouf.gabriel@chru-strasbourg.fr |
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| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Service d'Oncologie - CHU de Strasbourg - France | Recruiting | Strasbourg | 67091 | France |
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| ID | Term |
|---|---|
| D054973 | Perivascular Epithelioid Cell Neoplasms |
| D018207 | Angiomyolipoma |
| D018192 | Lymphangioleiomyomatosis |
| ID | Term |
|---|---|
| D018204 | Neoplasms, Connective and Soft Tissue |
| D009370 | Neoplasms by Histologic Type |
| D009369 | Neoplasms |
| D018205 | Neoplasms, Adipose Tissue |
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| D008203 | Lymphangiomyoma |
| D018190 | Neoplasm, Lymphatic Tissue |
| D008232 | Lymphoproliferative Disorders |
| D008206 | Lymphatic Diseases |
| D006425 | Hemic and Lymphatic Diseases |
| D007160 | Immunoproliferative Disorders |
| D007154 | Immune System Diseases |