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



Aggressive Angiomyxoma – A rare locally aggressive soft tissue tumour


Aggressive angiomyxoma is a locally aggressive mesenchymal tumour. 

This tumour usually occurs in women of reproductive age and is located in the perineal or pelvic region.

Histologically, this enigmatic soft tissue tumour is characterized by a prominent myxoid matrix and numerous thin-walled blood vessels.



Myxofibrosarcoma – A malignant neoplasm of fibroblastic origin [Pathology Infographic]


Myxofibrosarcoma, is the most common soft tissue sarcoma in the extremities of the elderly patients ( between sixty and eighty years of age).

Histopathologically, the tumour is characterized by spindle cellular proliferation with moderate cellular density in fibromyxoid stroma.

Source: De Castro BAC, Piancastelli ACC, Meyer RLB, Piancastelli PM, Ribeiro CA, Miranda RMC. Myxofibrosarcoma – Case report. Anais Brasileiros de Dermatologia. 2016;91(1):97-99. doi:10.1590/abd1806-4841.20163922.



Myxoinflammatory Fibroblastic Sarcoma: A distinct soft tissue tumour of low-grade malignancy [Pathology Infographic]


Myxoinflammatory fibroblastic sarcoma is a rare, painless, and intermediate (rarely metastasizing) fibroblastic tumor, which commonly occurs in the extremities, with an equal sex predilection.

This sarcoma is composed of a mixed inflammatory infiltrate along with spindled, epithelioid, and bizarre tumor cells in a background of hyaline and myxoid areas.

In spite of such a distinctive morphology, the tumor can be a diagnostic challenge, simulating inflammatory conditions as well as neoplastic nature.

For accurate diagnosis, the tumor requires extensive clinical, radiological, and pathological investigations.