Dermatofibrosarcoma protuberans is a very rare tumour that accounts for less than 0.1% of all malignant neoplasms. It is a locally aggressive tumour with high recurrence rate. Head and neck involvement is very uncommon and hence diverse treatment protocols have been recommended for its management in the currently available literature. We present a case of dermatofibrosarcoma protuberans over the lower part of right cheek in a middle aged male, which was successfully treated with wide local excision and reconstruction along with post-op radiotherapy. The patient was followed-up for 18 months with no recurrence. Prompt and accurate diagnosis along with multidisciplinary treatment is crucial for optimal management of this rare tumor. The optimal treatment option is surgical resection with wide margins along with adjuvant radiotherapy, which leads to better outcomes even in patients with positive surgical margins. Larger clinical trials with Imatinib will firmly establish its chemotherapeutic role in its management. Lifelong regular follow-up is essential for early detection of tumor.
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http://dx.doi.org/10.1007/s12070-021-02790-8 | DOI Listing |
J Craniofac Surg
October 2024
Department of Pathology, Pathohistology and Medical Cytology, University Clinical Center of Serbia, Belgrade, Serbia.
Introduction: Sarcomas are relatively rare malignant tumors of mesenchymal origin, representing only about 1% of tumors in the head and neck region.
Materials And Methods: A retrospective study involved patients with sarcomas of the head and neck region who were diagnosed and treated over a 5-year period.
Results: Nine patients were included, 4 men and 5 women.
Diseases
November 2024
Department of Gynecology and Gynecologic Oncology, Medical University of Białystok, Skłodowskiej Str. 24A, 15-276 Białystok, Poland.
Background: Dermatofibrosarcoma protuberans (DFSP) is a rare proliferative condition representing skin sarcomas known to locally recur yet very rarely known to metastasize. A typical characteristic is trunk localization, making vulvar occurrences an exception. Complete resection is the primary treatment.
View Article and Find Full Text PDFDermatopathology (Basel)
November 2024
Department of Dermatology, Louisiana State University Health Sciences Center, New Orleans, LA 70112, USA.
Spindle cell lipoma (SCL) is a benign adipocytic tumor usually found in the subcutis of the posterior neck, upper back, and shoulder, predominantly in middle-aged males. This case report describes an atypical presentation of SCL in a 26-year-old male with a history of malignant melanoma. The patient presented with an erythematous plaque with central hyperpigmentation on the right upper arm, an uncommon location and presentation for SCL.
View Article and Find Full Text PDFGeorgian Med News
October 2024
1Onkoderma - Clinic for Dermatology, Venereology and Dermatologic Surgery, Sofia, Bulgaria.
Dermatofibrosarcoma protuberans (DFSP) is a rare, low-grade cutaneous sarcoma typically found on the proximal extremities and the trunk, characterized by infiltrative growth and low risk of metastasis. High rates of local recurrence or relatively large tumor sizes can significantly complicate therapeutic management, particularly when 1) surgical intervention is not adequately performed and /or 2 access to newer medications is limited or their high cost imposes a financial burden on patients. We present the case of a 63-year-old male with a histologically confirmed dermatofibrosarcoma protuberans, measuring 6 cm in diameter, located on the right dorsal region, accompanied by several confluent multifocal nodules situated infralaterally to the primary formation.
View Article and Find Full Text PDFRadiol Case Rep
February 2025
Department of Adult Cardiac Surgery, University Medical Center Ho Chi Minh City, Ho Chi Minh, Vietnam.
Triple primary malignancies are rare, involving the occurrence of three distinct, unrelated cancers in a single patient, requiring a personalized, multidisciplinary treatment approach. A 69-year-old male was diagnosed with dermatofibrosarcoma protuberans (DFSP), lung adenocarcinoma, and papillary thyroid carcinoma. After amputation for DFSP, following staging and genetic evaluation, a multidisciplinary tumor board designed a tailored treatment plan, adjuvant chemotherapy for lung adenocarcinoma while monitor regularly the thyroid carcinoma via ultrasound.
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