Expression of dog1 in low-grade fibromyxoid sarcoma: A study of 19 cases and review of the literature

Benítez, A.; Rodríguez-Zarco, Enrique; Carrasco, Sara Pabón; Pereira-Gallardo, Sofia; Molina, Javier Brugal; García-Escudero, Antonio; Frías, A Robles; Marcilla, David; González‐Cámpora, Ricardo

doi:10.1016/j.anndiagpath.2017.05.002

Cited by 10 publications

(2 citation statements)

References 24 publications

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“…Although DOG1 is a sensitive and specific marker for GIST, its expression is reported in up to 94.7% of LGFMS. More specifically, nearly 40% cases of abdomen or retroperitoneum LGFMS have variable staining positivity of DOG1 [35][36][37]. It should be emphasized that the differential diagnosis between these two entities relies largely on appropriate molecular profiling: LGFMS expresses MUC4 protein and harbors characteristic gene fusions.…”

Section: Discussionmentioning

confidence: 99%

Primary small intestine mesenteric low-grade fibromyxoid sarcoma with foci of atypical epithelioid whorls and diffuse DOG1 expression: a case report

2020

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Background Low-grade fibromyxoid sarcoma (LGFMS) is a rare fibroblastic tumor often involving deep tissue of trunk and lower extremities in young to middle-aged patients. Rarely, LGFMS can occur in other sites including head and neck, chest, abdomen and female reproductive system. Three cases of LGFMS in mesentery of small intestine have been reported and all have conventional histologic features. Herein we reported a unique case of LGFMS in mesentery of small intestine. Case presentation A 43 year-old male with chief complaint of lower back pain for 4 years presented to our hospital. Physical exam reveal a firm, non-tender, non-distended, mobile large abdominal mass, which was shown on abdominal CT as a 10 cm retroperitoneal tumor. Biopsy revealed a spindle cell neoplasm in a myxoid background with a delicate vascular network. Tumor resection was performed. Gross examination of the resected specimen showed a 10.8 cm, tan-white, smooth, firm, lobulated mesenteric mass with bulging and gelatinous cut surface and confined within small bowel serosa. Microscopic examination demonstrated foci epithelioid cords and whorls with prominent atypia, in additional of regular, bland-appearing spindle cells in a fibrous and myxoid stroma and osseous metaplasia. The tumor cells stained diffusely positive for DOG1 with moderate staining density, and diffusely and strongly positive for MUC4. Rearrangement involving FUS (16p11.2) gene was identified with break-apart probe and confirmed by Anchored Multiplex PCR. A final diagnosis of low-grade fibromyxoid sarcoma was rendered. Conclusion Our case highlights the importance of including LGFMS in the differential diagnosis of mesenteric tumors and the DOG1 positivity which could represent a potential diagnostic pitfall.

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Section: Discussionmentioning

confidence: 99%

Primary small intestine mesenteric low-grade fibromyxoid sarcoma with foci of atypical epithelioid whorls and diffuse DOG1 expression: a case report

2020

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“…Involvement of acral sites is not highly emphasized in the literature. After encountering this case, we reviewed the literature and found 24 cases (mostly embedded in case series) of LGFMS involving distal extremities and acral sites (Table ) …”

Section: Discussionmentioning

confidence: 99%

Low‐grade fibromyxoid sarcoma of acral sites: Case report and literature review

Saab‐Chalhoub

Al‐Rohil

2019

J Cutan Pathol

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Low-grade fibromyxoid sarcoma (LGFMS) is a rare soft tissue sarcoma that usually presents as a deep-seated tumor in young adults; however, they can occur on superficial sites, mostly documented in pediatric age groups.LGFMS presenting on acral sites is not highly emphasized in the general pathology or dermatopathology literature. The case presented is that of a 30-year-old man with a foot mass that was removed 15 years earlier and subsequently recurred as two masses, the first occurring between the third and fourth toes/metatarsal region and the second over the lateral tarsal region. An excisional biopsy showed a relatively circumscribed, bland spindle cell proliferation with hypocellular and hypercellular zones. The cells showed minimal pleomorphism and lacked mitotic activity. Immunohistochemical analysis showed immunoreactivity for MUC4 and break-apart fluorescence in situ hybridization was positive for FUS rearrangement, confirming the diagnosis of LGFMS. There are multiple spindle cell tumors that occur on acral sites which usually generates a list of differential diagnoses; however, LGFMS is not usually discussed in that anatomic location. Awareness of the occurrence of LGFMS on acral sites is important to avoid misdiagnosis of this deceptively benign-appearing tumor.

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Low Grade Fibromyxoid Sarcoma

Bovée¹

2019

Encyclopedia of Pathology

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Expression of dog1 in low-grade fibromyxoid sarcoma: A study of 19 cases and review of the literature

Cited by 10 publications

References 24 publications

Primary small intestine mesenteric low-grade fibromyxoid sarcoma with foci of atypical epithelioid whorls and diffuse DOG1 expression: a case report

Primary small intestine mesenteric low-grade fibromyxoid sarcoma with foci of atypical epithelioid whorls and diffuse DOG1 expression: a case report

Low‐grade fibromyxoid sarcoma of acral sites: Case report and literature review

Low Grade Fibromyxoid Sarcoma

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