Adult perineal sarcomas.
OBJECTIVE
Primary perineal sarcoma in adults is a rare disease that has only been documented to occur in isolated case reports.


METHODS
To better characterize and define the natural history of perineal sarcoma in adults (> or = 18 years), we reviewed our experience with treatment of perineal sarcoma between 1982 and 1999 (nine cases).


RESULTS
Epithelioid sarcoma (n = 4) was the most common histologic subtype. Seven cases (78%) were histologically high grade, and lesions were most commonly < 5 cm. All patients were treated with wide local excision. External beam radiation was the most commonly used form of adjuvant therapy (n = 6). Recurrences were noted in five patients, and the recurrences were most commonly local (60%). Median time to first recurrence was 21 months. Six of nine patients are alive with a median follow-up of 54 months. Three died of recurrent/metastatic disease at 16, 51, and 54 months after initial surgery.


CONCLUSIONS
Aggressive therapy and follow-up beginning with wide excision can be associated with long-term survival in adults with primary perineal sarcoma.
Grobmyer
S R
SR
Department of Surgery, Memorial-Sloan Kettering Cancer Center, New York, New York, USA.
Clary
B
B
Lewis
J J
JJ
Delgado
R
R
Woodruff
J M
JM
Brennan
M F
MF
D000293:Adolescent; D000328:Adult; D000368:Aged; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009364:Neoplasm Recurrence, Local; D010502:Perineum / Q000601:surgery; D018714:Radiotherapy, Adjuvant; D012509:Sarcoma / Q000473:pathology / Q000532:radiotherapy / Q000601:surgery*; D012983:Soft Tissue Neoplasms / Q000473:pathology / Q000532:radiotherapy / Q000601:surgery*