Hyaline Fibromatosis Syndrome [Infantile Systemic Hyalinosis and Juvenile Hyaline Fibromatosis ] – Pathology Infographic

 

Hyaline fibromatosis syndrome is the current name for clinical manifestations of diseases previously known as “infantile systemic hyalinosis” and “juvenile hyaline fibromatosis”.

Infantile systemic hyalinosis and juvenile hyaline fibromatosis are very rare recessive autosomal hereditary diseases that arise from mutation of gene ANTXR2 (anthrax toxin receptor-2), also known as gene CMG2 (capillary morphogenesis gene2), located in chromosome 4q21.

Visit: Pathology of Juvenile Hyaline Fibromatosis

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Angiomyofibroblastoma [Pathology Infographic]

 

Angiomyofibroblastoma is a rare tumor that occurs in the genital tract, vulva, perineum, and pelvis of women.

In men, angiomyofibroblastoma-like tumors are known to occur in areas such as the inguinal area, scrotum, and perineum.

In 1997, Nucci et al first described an angiomyofibroblastoma-like tumor as a cellular angiofibroma .

Thereafter, in 1998, Laskin et al termed it an angiomyofibroblastoma-like tumor .

Clinically, the tumor has asymptomatic, well-circumscribed, and slowly growing characteristics.

 

 

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Pleomorphic Hyalinizing Angiectatic Tumour [Pathology Infographic]

 

Pleomorphic hyalinizing angiectatic tumors (PHATs) are non-metastasizing soft-tissue tumors of uncertain lineage occurring within the superficial subcutaneous tissues and muscles .

Presents in adults between the ages of 10 and 83 years (median age of 51 years), PHATs are more commonly observed in females than in males.

The majority of affected patients present with slowly-growing, painless masses, most commonly involving the lower extremities.

Rarer tumor sites include the arm, chest wall, axilla, popliteal fossa, buttocks, inguinal region, perineum, buccal mucosa and breast.

 

SUZUKI K, YASUDA T, HORI T, et al. Pleomorphic hyalinizing angiectatic tumor arising in the thigh: A case report. Oncology Letters. 2014;7(4):1249-1252. doi:10.3892/ol.2014.1883.

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