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Intraabdominal desmoplastic small round cell tumors: a diagnostic and
therapeutic challenge.Cancer.
2005 Sep 15;104(6):1264-70.
BACKGROUND:
Intraabdominal desmoplastic small round cell tumors (IDSRCT) are
uncommon but aggressive tumors that occur in young males. To the
authors' knowledge, only limited data are available on the natural
history and optimal treatment of this disease. METHODS: The authors
reviewed 12 patients with IDSRCT who were treated at their institution
between January 1991 and December 2001. RESULTS: All patients were
males, with a median age of 26 years. All patients were symptomatic at
the time of presentation, with a mean duration of symptoms of 2
months. Common presenting symptoms and signs were abdominal pain (67%
of patients), palpable abdominal mass (58% of patients), abdominal
distension (42% of patients), and hepatomegaly (33% of patients). Six
patients (50%) had distant metastases at presentation. Five patients
underwent biopsy only. Surgical resection was attempted in seven
patients and included macroscopic total resection in three patients
and debulking in four patients. All of those patients subsequently
developed recurrent or progressive disease, which required a second
operation in six patients. Overall, 6 patients (50%) developed
symptomatic intestinal obstruction requiring surgical management, and
3 patients (25%) developed ureteral obstruction. All 12 patients
received multiagent chemotherapy. Seven patients (55%) also received
radiation therapy. The median survival of patients who underwent
surgical resection was 34 months, whereas the median survival of
patients who underwent biopsy alone was 14 months. One patient
remained alive 72 months after he underwent complete resection of
primary and recurrent tumors, and 1 patient remained alive with
disease 32 months after he underwent complete resection of a primary
tumor. CONCLUSIONS: Patients with IDSRCT presented with a short
duration of nonspecific symptoms, and the disease was fatal almost
uniformly, regardless of the treatment modality used. Surgical
resection may prolong survival in some patients. |
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Desmoplastic small round cell
tumour
Visit: Soft Tissue Pathology
 |
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Results of multimodal treatment for desmoplastic small round cell
tumors.J
Pediatr Surg. 2005 Jan;40(1):251-5.
PURPOSE:
Desmoplastic small round cell tumors (DSRCTs) are rare aggressive
neoplasms that frequently present with large symptomatic
intraabdominal masses. We examined the effects of multimodal therapy
including induction chemotherapy, aggressive surgical debulking, and
external beam radiotherapy on patients with DSRCT. METHODS:
Institutional Review Board permission was obtained. Sixty-six
patients were diagnosed by histology, immunohistochemistry, and or
cytogenetics as having DSRCT at our institution from July 1, 1972,
to July 1, 2003. Data were collected on patient demographics,
presenting symptoms, tumor location and extent, treatment regimen,
and overall survival. RESULTS: A majority of patients were male
(91%), Caucasian (85%), and with a median age of 19 (7-58) years old
at diagnosis. The most common presenting complaint was an
intraabdominal mass (64%). In 63 patients (96%), the primary tumor
was located in the abdomen or pelvis. Thirty-three (50%) had
positive lymph nodes and 27 (41%) had distant parenchymal metastases
at diagnosis. Overall, 3- and 5-year survivals were 44% and 15%,
respectively. Twenty-nine of these patients (44%) underwent
induction chemotherapy (P6), surgical debulking, and radiotherapy.
Three-year survival was 55% in those receiving chemotherapy,
surgery, and radiotherapy vs 27% when all 3 modalities were not used
(P < .02). Gross tumor resection was highly significant in
prolonging overall survival; 3-year survival was 58% in patients
treated with gross tumor resection compared to no survivors past 3
years in the nonresection cohort (P < .00001). Ten patients (15%)
have no evidence of disease with a median follow-up of 2.4 years
(range, 0.4-11.2 years). CONCLUSIONS: Multimodal therapy results in
improved survival in patients with DSRCT. Aggressive surgical
resection of these extensive intraabdominal neoplasms correlates
with improved patient outcome. |
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February 2008
Myxoid
Tumours of Soft Tissue
Classification
of Soft Tissue Tumour
Gross examination of soft tissue specimen |
