Unusual Clinical Presentation of Clear Cell Sarcoma in a Young Woman

Clear cell sarcoma (CCS) is a rare but aggressive malignancy that typically occurs in young adults and is characterized by soft tissue tumors of the extremities. CCS can be difficult to distinguish from metastatic melanoma based solely on histology and immunohistochemistry (IHC) because of the signi...

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Veröffentlicht in:Oncology (Williston Park, N.Y.) N.Y.), 2023-10, Vol.37 (10), p.412-416
Hauptverfasser: Asif, Samia, Hurley, Brendan J, Haroon, Sehr, Lele, Subodh, Sharma, Bhavina
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container_end_page 416
container_issue 10
container_start_page 412
container_title Oncology (Williston Park, N.Y.)
container_volume 37
creator Asif, Samia
Hurley, Brendan J
Haroon, Sehr
Lele, Subodh
Sharma, Bhavina
description Clear cell sarcoma (CCS) is a rare but aggressive malignancy that typically occurs in young adults and is characterized by soft tissue tumors of the extremities. CCS can be difficult to distinguish from metastatic melanoma based solely on histology and immunohistochemistry (IHC) because of the significant overlap between them. However, it is imperative to get an accurate clinical diagnosis, as it informs disease staging and treatment options for patient care. Present in approximately 75% of CCS cases, the EWSR1 gene rearrangement detected by fluorescence in situ hybridization (FISH) can help with establishing a diagnosis; the underlying reciprocal translocation has never been reported in cutaneous melanoma. We reviewed a case of a young woman who presented with a confusing picture of widespread lymphadenopathy, cutaneous metastases, and electrolyte derangements and was subsequently diagnosed with metastatic CCS.This case suggests possible value in performing molecular testing when a clinical picture does not correspond with what is expected for melanoma. It also raises the question of whether CCS cases may be underreported. This case highlights an uncommon presentation that may not be recognized as a manifestation of CCS by an oncologist who is not a sarcoma specialist. It is unclear how COVID-19 vaccination contributed to her clinical presentation, and it is also unclear whether an early diagnosis would have changed her clinical outcome.
doi_str_mv 10.46883/2023.25921005
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source MEDLINE; EZB-FREE-00999 freely available EZB journals
subjects Abdomen
Antibiotics
Antimitotic agents
Antineoplastic agents
Biopsy
Bone marrow
COVID-19
COVID-19 Vaccines
Cytogenetics
Development and progression
Disodium pamidronate
Female
Health aspects
Humans
Hydronephrosis
Hypercalcemia
Immunohistochemistry
Immunotherapy
In Situ Hybridization, Fluorescence
Kidneys
Lymphatic system
Lymphoma
Medical imaging
Melanoma
Metastasis
Ovaries
Rasburicase
Sarcoma
Sarcoma, Clear Cell - diagnosis
Sarcoma, Clear Cell - genetics
Skin cancer
Skin Neoplasms - diagnosis
Skin Neoplasms - genetics
Soft Tissue Neoplasms
Vaccination
Young Adult
Young adults
title Unusual Clinical Presentation of Clear Cell Sarcoma in a Young Woman
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