1. Atypical fibrous histiocytoma of the skin with CD30 and p80/ALK1 positivity and ALK gene rearrangement.
- Author
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Szablewski V, Laurent-Roussel S, Rethers L, Rommel A, Van Eeckhout P, Camboni A, Willocz P, Copie-Bergman C, and Ortonne N
- Subjects
- Activin Receptors, Type II genetics, Adult, Diagnosis, Differential, Female, Histiocytoma, Benign Fibrous genetics, Histiocytoma, Benign Fibrous metabolism, Humans, Male, Skin metabolism, Skin pathology, Skin Neoplasms genetics, Skin Neoplasms metabolism, Thigh pathology, Young Adult, Activin Receptors, Type II metabolism, Gene Rearrangement, Histiocytoma, Benign Fibrous pathology, Ki-1 Antigen metabolism, Skin Neoplasms pathology
- Abstract
We report the case of a two patients who presented with a solitary, asymptomatic, angiomatoid nodule on the right thigh. Histopathological finding showed a poorly circumscribed lesion, located in the dermis. The morphological aspect strongly suggested the diagnosis of atypical fibrous histiocytoma (AFH), but surprisingly, the neoplastic cells were diffusely CD30+, with a membrane staining devoid of paranuclear dot. The lesions were tested for p80/ALK1 expression. Surprisingly, we found a diffuse cytoplasmic positivity. Interestingly, using break-apart fluorescent in situ hybridization (FISH), we evidenced an ALK rearrangement in nearly 50% of the neoplastic cells. The expression of CD30 and ALK1 with ALK gene rearrangement raised the possibility of three diagnoses: a primary cutaneous anaplastic large cell lymphoma (ALCL), a cutaneous inflammatory myofibroblastic tumor (IMT), an AFH of the skin associated with ALK gene rearrangement and CD30 positivity. The three hypotheses were discussed and finally, although p80/ALK1 expression and cytogenetic abnormalities in fibrous histiocytoma (FH) are not yet reported to the best of our knowledge, we favored the diagnosis of AFH., (© 2014 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)
- Published
- 2014
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