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Local ALK-Positive Histiocytosis With Unusual Morphology and Novel TRIM33-ALK Gene Fusion.
- Source :
-
International journal of surgical pathology [Int J Surg Pathol] 2021 Aug; Vol. 29 (5), pp. 543-549. Date of Electronic Publication: 2020 Nov 27. - Publication Year :
- 2021
-
Abstract
- ALK-positive histiocytosis was first described in 2008 as a systemic histiocytic disorder involving young infants and neonates. Subsequently, cases of local ALK-positive histiocytosis as well as clinical presentation in adult patients have been increasingly reported in the literature. The current case documented the hitherto largest local ALK-positive histiocytosis lesion involving the mesentery of a 20-year-old female patient, a clinical presentation that has not been previously reported in the medical literature. Of note was the presence of numerous lymphocytes, plasma cells, and eosinophils as well as the formation of lymphoid follicles in the lesion, mimicking an inflammatory myofibroblastic tumor. Other unique histologic aspects of the current case included the nested arrangement of the histiocytes, intravascular extension of the histiocytic proliferation into a large vein, and tumor necrosis. Notably, molecular studies revealed a novel TRIM33 (exon 12) -ALK (exon 20) gene fusion. Therefore, ALK-positive histiocytosis with TRIM33-ALK gene fusion expands the clinical, histologic, and molecular spectrum of local ALK-positive histiocytosis. Since ALK-positive histiocytosis associated with a significant inflammatory component can pose considerable diagnostic challenges, increased awareness of this peculiar variant of ALK-positive histiocytosis is essential to minimize the risk of misdiagnosis.
- Subjects :
- Female
Histiocytosis genetics
Histiocytosis pathology
Histiocytosis surgery
Humans
Mesentery diagnostic imaging
Mesentery surgery
Tomography, X-Ray Computed
Young Adult
Anaplastic Lymphoma Kinase genetics
Histiocytosis diagnosis
Mesentery pathology
Oncogene Proteins, Fusion genetics
Transcription Factors genetics
Subjects
Details
- Language :
- English
- ISSN :
- 1940-2465
- Volume :
- 29
- Issue :
- 5
- Database :
- MEDLINE
- Journal :
- International journal of surgical pathology
- Publication Type :
- Academic Journal
- Accession number :
- 33243034
- Full Text :
- https://doi.org/10.1177/1066896920976862