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Low-grade dermatofibrosarcoma protuberance - A rare case report.

T L Yogesh¹, Anjani Kumar Jha², Sindhumati Jayaraman¹

¹Department of Oral Pathology and Microbiology, Rajiv Gandhi College of Dental Sciences and Research Centre, Hebbal, Bengaluru, Karnataka, India.

Journal of Oral and Maxillofacial Pathology : JOMFP

|August 19, 2024

Summary

This summary is machine-generated.

Dermatofibrosarcoma protuberans (DFSP), a rare soft-tissue tumor, can present in the head and neck. This case highlights the importance of accurate diagnosis due to its potential for recurrence.

Keywords:

CD34 Cartwheel DFSP dermatofibrosarcoma protuberans storiform

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Area of Science:

Oncology
Dermatopathology
Surgical Pathology

Background:

Dermatofibrosarcoma protuberans (DFSP) is a rare, slow-growing soft-tissue neoplasm.
DFSP commonly affects the trunk and extremities, with head and neck involvement being less frequent.
While DFSP has low metastatic potential, it exhibits a high local recurrence rate.

Observation:

A case of a female patient with a large soft tissue growth on the right cheek, chin, and neck is presented.
Initial clinical impression suggested a benign lesion like a lipoma or sebaceous cyst.
Histological examination revealed bland spindle cells in a storiform pattern, prompting further investigation.

Findings:

Immunohistochemistry (IHC) confirmed the diagnosis of Dermatofibrosarcoma protuberans (DFSP).
The tumor's location in the head and neck region is uncommon.
The initial presentation mimicked benign conditions, leading to a potential diagnostic challenge.

Implications:

This case underscores the importance of considering DFSP in the differential diagnosis of head and neck soft tissue masses.
Accurate histological and IHC evaluation is crucial for correct diagnosis and management of DFSP.
Early and precise diagnosis is vital to prevent local recurrence and ensure appropriate treatment for this rare tumor.