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Dermatofibrosarcoma protuberans with fibrosarcomatous transformation of the head and neck.

Angouridakis N, Kafas P, Jerjes W, Triaridis S, Upile T, Karkavelas G, Nikolaou A - Head Neck Oncol (2011)

Bottom Line: Dermatofibrosarcoma protuberans (DFSP) is a rare cutaneous neoplasm associated with a high cure rate.A 28-year-old Mediterranean female presented with a 45-day history of rapidly growing cutaneous lesion of the face.Subsequently, the patient underwent wide local surgical resection, followed by reconstruction.

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Affiliation: Otorhinolaryngology, Head and Neck Surgery Department, AHEPA University Hospital, Thessaloniki, Greece. nikos.angouridakis@gmail.com

ABSTRACT
Dermatofibrosarcoma protuberans (DFSP) is a rare cutaneous neoplasm associated with a high cure rate. We present a case of aggressive DFSP with fibrosarcomatous areas in the head and neck. A 28-year-old Mediterranean female presented with a 45-day history of rapidly growing cutaneous lesion of the face. Surgical biopsy confirmed the diagnosis of DFSP. Subsequently, the patient underwent wide local surgical resection, followed by reconstruction. Histopathology report revealed fibrosarcomatous transformation and the patient underwent adjuvant radiotherapy. The patient continues to be disease free at the 35-month follow-up.Although DFSP behave as non-aggressive malignancy, surgery with complete removal of the affected area is the intervention of choice. Moreover, adjuvant treatment and follow-up of the patient is essential in order to prevent recurrence.

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Histopathology image showing immunopositivity to CD34. (a) ×25 showing immunopositivity to CD34. (b) ×40 showing immunopositivity to CD34.
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Figure 6: Histopathology image showing immunopositivity to CD34. (a) ×25 showing immunopositivity to CD34. (b) ×40 showing immunopositivity to CD34.

Mentions: Histopathological examination, of the completely excised tumour, revealed a nodular neoplastic lesion composed of large spindle-shaped cells with prominent nuclei, showing a high mitotic index and areas of necrosis, infiltrating into the subcutaneous fat and muscles (Figure 5). Further immunohistochemical analysis revealed tumour cells which were positive for CD34, and c-kit antigen. A few cells were positive for SMA and CD68. Staining for Ki67/MIB 1 showed 50% positive cells and increased mitotic rate (>20/10 HPFs). It was concluded that this constellation of staining represented a "dermatofibrosarcoma protuberans with fibrosarcomatous transformation" (Figure 6).


Dermatofibrosarcoma protuberans with fibrosarcomatous transformation of the head and neck.

Angouridakis N, Kafas P, Jerjes W, Triaridis S, Upile T, Karkavelas G, Nikolaou A - Head Neck Oncol (2011)

Histopathology image showing immunopositivity to CD34. (a) ×25 showing immunopositivity to CD34. (b) ×40 showing immunopositivity to CD34.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3038985&req=5

Figure 6: Histopathology image showing immunopositivity to CD34. (a) ×25 showing immunopositivity to CD34. (b) ×40 showing immunopositivity to CD34.
Mentions: Histopathological examination, of the completely excised tumour, revealed a nodular neoplastic lesion composed of large spindle-shaped cells with prominent nuclei, showing a high mitotic index and areas of necrosis, infiltrating into the subcutaneous fat and muscles (Figure 5). Further immunohistochemical analysis revealed tumour cells which were positive for CD34, and c-kit antigen. A few cells were positive for SMA and CD68. Staining for Ki67/MIB 1 showed 50% positive cells and increased mitotic rate (>20/10 HPFs). It was concluded that this constellation of staining represented a "dermatofibrosarcoma protuberans with fibrosarcomatous transformation" (Figure 6).

Bottom Line: Dermatofibrosarcoma protuberans (DFSP) is a rare cutaneous neoplasm associated with a high cure rate.A 28-year-old Mediterranean female presented with a 45-day history of rapidly growing cutaneous lesion of the face.Subsequently, the patient underwent wide local surgical resection, followed by reconstruction.

View Article: PubMed Central - HTML - PubMed

Affiliation: Otorhinolaryngology, Head and Neck Surgery Department, AHEPA University Hospital, Thessaloniki, Greece. nikos.angouridakis@gmail.com

ABSTRACT
Dermatofibrosarcoma protuberans (DFSP) is a rare cutaneous neoplasm associated with a high cure rate. We present a case of aggressive DFSP with fibrosarcomatous areas in the head and neck. A 28-year-old Mediterranean female presented with a 45-day history of rapidly growing cutaneous lesion of the face. Surgical biopsy confirmed the diagnosis of DFSP. Subsequently, the patient underwent wide local surgical resection, followed by reconstruction. Histopathology report revealed fibrosarcomatous transformation and the patient underwent adjuvant radiotherapy. The patient continues to be disease free at the 35-month follow-up.Although DFSP behave as non-aggressive malignancy, surgery with complete removal of the affected area is the intervention of choice. Moreover, adjuvant treatment and follow-up of the patient is essential in order to prevent recurrence.

Show MeSH
Related in: MedlinePlus