5 research outputs found
Desmoplastic small round cell tumor: Extra abdominal and abdominal presentations and the results of treatment
BACKGROUND: Desmoplastic small round cell tumor (DSRCT) is a rare
malignant neoplasm of adolescent males. Current multimodality treatment
prolongs life and rarely achieves cure. Aim : To review the presenting
features, histopathology and outcome of 18 patients with DSRCT treated
at a single institution. Setting and Design : This is a retrospective
observational study of patients with DSRCT who presented at the Tata
Memorial Hospital between January 1994 to January 2005. Materials and
Methods: Eighteen patients of DSRCT seen during this period were
evaluated for their clinical presentation, response to chemotherapy and
other multimodality treatment and overall survival. The cohort of 18
patients included 11 males (61%) and 7 females (39%) with a mean age of
16 years (Range 1\ubd - 30 years). Majority (83%) presented with
abdomino-pelvic disease. The others, involving chest wall and
extremities. There were 6 patients (33%) with metastatic disease at
presentation. Results: The treatment primarily included a
multimodality approach using a combination of multiagent chemotherapy
with adjuvant surgery and radiotherapy as applicable. A response rate
of 39% (CR-1, PR-6), with chemotherapy was observed. The overall
response rate after multimodality treatment was 39% (CR-5, PR-2). The
overall survival was poor except in patients who had complete excision
of the tumor. Conclusion: Abdomino-pelvic site was the commonest
presentation, the disease can occur at other non-serosal surfaces also.
Despite aggressive treatment the outcome was poor. However, complete
surgical excision seems to provide a better survival
Desmoplastic small round cell tumor: Extra abdominal and abdominal presentations and the results of treatment
BACKGROUND: Desmoplastic small round cell tumor (DSRCT) is a rare
malignant neoplasm of adolescent males. Current multimodality treatment
prolongs life and rarely achieves cure. Aim : To review the presenting
features, histopathology and outcome of 18 patients with DSRCT treated
at a single institution. Setting and Design : This is a retrospective
observational study of patients with DSRCT who presented at the Tata
Memorial Hospital between January 1994 to January 2005. Materials and
Methods: Eighteen patients of DSRCT seen during this period were
evaluated for their clinical presentation, response to chemotherapy and
other multimodality treatment and overall survival. The cohort of 18
patients included 11 males (61%) and 7 females (39%) with a mean age of
16 years (Range 1½ - 30 years). Majority (83%) presented with
abdomino-pelvic disease. The others, involving chest wall and
extremities. There were 6 patients (33%) with metastatic disease at
presentation. Results: The treatment primarily included a
multimodality approach using a combination of multiagent chemotherapy
with adjuvant surgery and radiotherapy as applicable. A response rate
of 39% (CR-1, PR-6), with chemotherapy was observed. The overall
response rate after multimodality treatment was 39% (CR-5, PR-2). The
overall survival was poor except in patients who had complete excision
of the tumor. Conclusion: Abdomino-pelvic site was the commonest
presentation, the disease can occur at other non-serosal surfaces also.
Despite aggressive treatment the outcome was poor. However, complete
surgical excision seems to provide a better survival
Case Report - Infantile fibromatosis of scrotum
Infantile fibromatosis is a benign but locally aggressive tumour, the
tumour presenting as asymptomatic, firm and solid mass. The most
frequent types are the infantile fibromatosis (head, neck, shoulder,
upper arm or thigh), extra-abdominal fibromatosis (chest wall, back and
thigh) and fibromatosis colli (neck), but only rarely has it been
reported to involve the external genitalia or scrotum. We report a case
of infantile fibromatosis of scrotum in a 1-year-old child