Angiolipomatosis, Familial

Last update 01 Nov 2024

Basic Info

Synonyms

ANGIOLIPOMA MICROTHROMBOTICUM, ANGIOLIPOMATOSIS, FAMILIAL, Angiolipoma Microthromboticum

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Introduction

A rare genetic subcutaneous tissue disorder with the presence of benign usually multiple subcutaneous tumours. The tumours are composed of adipose tissue and blood vessels typically manifesting as yellow firm circumscribed 1-4 cm in diameter tumours located in the arms, legs and trunk with deep extension of the lesions between muscles, tendons and joint capsules (without infiltration of these structures) in several members of a single family. Tumours may be tender or mildly painful when palpated and do not regress spontaneously.

100 Clinical Results associated with Angiolipomatosis, Familial

100 Translational Medicine associated with Angiolipomatosis, Familial

0 Patents (Medical) associated with Angiolipomatosis, Familial

Literatures (Medical) associated with Angiolipomatosis, Familial

01 Jun 2024·Annales d'Endocrinologie

Lipomatoses

Review

Author: Dupuis, Hippolyte ; Espiard, Stéphanie ; Douillard, Claire ; Lemaitre, Madleen ; Vantyghem, Marie-Christine ; Jannin, Arnaud

Lipomatoses are benign proliferation of adipose tissue. Lipomas (benign fat tumors) are the most common component of lipomatosis. They may be unique or multiple, encapsulated or not, subcutaneous or sometimes visceral. In some cases, they form large areas of non-encapsulated fat hypertrophy, with a variable degree of fibrosis. They can develop despite the absence of obesity. They may be familial or acquired. At difference with lipodystrophy syndromes, they are not associated with lipoatrophy areas, except in some rare cases such as type 2 familial partial lipodystrophy syndromes (FPLD2). Their metabolic impact is variable in part depending on associated obesity. They may have functional or aesthetic consequences. Lipomatosis may be isolated, be part of a syndrome, or may be visceral. Isolated lipomatoses include multiple symmetrical lipomatosis (Madelung disease or Launois-Bensaude syndrome), familial multiple lipomatosis, the painful Dercum's disease also called Adiposis Dolorosa or Ander syndrome, mesosomatic lipomatosis also called Roch-Leri lipomatosis, familial angiolipomatosis, lipedema and hibernomas. Syndromic lipomatoses include PIK3CA-related disorders, Cowden/PTEN hamartomas-tumor syndrome, some lipodystrophy syndromes, and mitochondrial diseases, especially MERRF, multiple endocrine neoplasia type 1, neurofibromatosis type 1, Wilson disease, Pai or Haberland syndromes. Finally, visceral lipomatoses have been reported in numerous organs and sites: pancreatic, adrenal, abdominal, epidural, mediastinal, epicardial… The aim of this review is to present the main types of lipomatosis and their physiopathological component, when it is known.

01 Sep 2019·Asian Journal of Neurosurgery

Exploring a tumor spectrum in a patient with familial angiolipomatosis

Author: Maheshwari, Shradha ; Arora, Eham

Angiolipomas are uncommon spinal tumors which differ from their cutaneous counterparts in having larger caliber vascular stroma. Although slow growing, they can cause rapid spinal cord compression and sudden-onset sensorimotor symptoms due to vascular engorgement, hemorrhage, or thrombosis. The goal of surgery is spinal decompression, and favorable outcome is the rule. We report a patient with spinal angiolipoma, vertebral hemangioma along with subcutaneous lipomas and angiolipomas, exhibiting the entire histopathological spectrum of these related soft-tissue tumors. Analysis of his family tree revealed a hereditary predilection. Familial angiolipomatosis is an uncommon genetic condition which has not been reported to occur with spinal angiolipomas thus far.

01 Jan 2015·CutisQ4 · MEDICINE

Autosomal-dominant familial angiolipomatosis.

Q4 · MEDICINE

Article

Author: Andea, Aleodor A ; Garib, George ; Siegal, Gene P

Angiolipomas are among the most common benign soft-tissue tumors and usually present as solitary nodules; however, angiolipomas also may present as multiple subcutaneous nodules, typically on the arms and trunk of young men. Although multiple angiolipomas most often occur sporadically, a family history can be identified in a minority of cases. Familial angiolipomatosis is a rare condition with an autosomal-recessive transmission pattern that is characterized by multiple subcutaneous tumors and a family history of similar lesions, which are not associated with malignant neoplasms. We report a case of familial angiolipomatosis with an unusual autosomal-dominant transmission pattern. Our patient presented with multiple angiolipomas that were highly suggestive of familial angiolipomatosis transmitted in an autosomal-dominant fashion, as he had several family members with a history of similar fatty tumors. Autosomal-dominant familial angiolipomatosis may be misdiagnosed as neurofibromatosis type I. Therefore, in cases of multiple subcutaneous tumors and a family history of similar lesions, histologic examination is important to establish the correct diagnosis.

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Angiolipomatosis, Familial

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