Diagnosis
Based on their clinical presentation and gross appearance IMF tumors can be confused with a wide range of benign and malignant papular, nodular, and tumorous lesions particular in cases which involve multiple tumors. However, the presence of two zone histopathology, family history of disease, and presence of the above cited PDGFRB gene mutations are almost always definitive indicators of IMF. IMF and the classic form of mesoblastic nephroma have been suggested to be the same disease because of their very similar histopathology. However, mesoblastic nephroma tumor cells, unlike IMF tumor cells, express cyclin D1 and Beta-catenin proteins and therefore likely have a very different cellular origin than IMF tumors. Infantile digital fibromatosis, a tumor that develops primarily in the fingers and toes, had been regarded as a type of IMF. However its tumors have a distinctly different clinical presentation and histopathology than IMF. The World Health Organization (2020) redefined infantile digital fibromatosis as a benign tumor in the in the category of benign fibroblastic and myofibroblastic tumors and therefore different than IMF. Treatment and prognosis
There are no controlled studies to define the optimal treatment(s) for IMF. The following give the currently widely used and recommended treatments along with the result of these treatments for the three categories of the IMF tumors. Solitary IMF tumors
Solitary IMF tumors typically grow slowly, produce few or no symptoms, and often regress spontaneously within 18 to 24 months of diagnosis. A watchful waiting strategy is commonly used for these tumors.
Woolly hair nevus (alternatively spelled "Wooly hair nevus") is a congenital condition in which hair in a circumscribed area of the scalp is kinked or woolly. See also
Woolly hair
Naxos syndrome
Striate palmoplantar keratoderma, woolly hair, and left ventricular dilated cardiomyopathy
List of cutaneous conditions

References


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