Cutaneous inflammatory myofibroblastic tumor with CARS-ALK fusion: Case report and literature review

J Cutan Pathol. 2022 Sep;49(9):795-801. doi: 10.1111/cup.14261. Epub 2022 Jun 6.

Abstract

Cutaneous inflammatory myofibroblastic tumors (IMT) constitute a rare entity, generating a diagnostic pitfall when diagnosing spindle cell proliferation within the dermis. Raising awareness of this tumor among dermatopathologists remains vital in differentiating it from common cutaneous tumors such as fibrous histiocytoma, atypical fibroxanthoma, melanoma, poorly differentiated carcinoma, and other more aggressive tumors. Accurate diagnosis of IMT aids in ensuring appropriate management and follow-up for patients while preventing unnecessary harm and overtreatment. Here we report a case of a 38-year-old female with a painless, slow-growing nodule of the left posterior scalp initially diagnosed as a dermatofibroma. The histopathological examination revealed an ill-defined dermal nodule of spindled cells without connection or infiltration of the epidermis. At high power, the cells were arranged in fascicles with a prominent background of lymphocytic infiltrate. Immunohistochemical analysis showed strong diffuse immunoreactivity for anaplastic lymphoma kinase (ALK), and targeted RNA sequencing identified a CARS-ALK fusion ultimately confirming the accurate diagnosis of a cutaneous IMT.

Keywords: ALK; cutaneous spindle cell tumors; inflammatory myofibroblastic tumor.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adult
  • Anaplastic Lymphoma Kinase / genetics
  • Female
  • Gene Fusion
  • Granuloma, Plasma Cell* / diagnosis
  • Granuloma, Plasma Cell* / genetics
  • Humans
  • Skin Neoplasms* / diagnosis
  • Skin Neoplasms* / genetics

Substances

  • Anaplastic Lymphoma Kinase