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Desmoplastic Small Round Cell Tumor of the Abdomen: a Case Report and Literature Review of Therapeutic Options

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Desmoplastic Small Round Cell Tumor of the Abdomen: a Case Report and Literature Review of Therapeutic Options Vol.4, No.4, 207-211 (2012) Health http://dx.doi.org/10.4236/health.2012.44031 Desmoplastic small round cell tumor of the abdomen: A case report and literature review of therapeutic options Hafida Benhammane1*, Leila Chbani2, Abdelmalek Ousadden3, Ouadii Mouquit3, Siham Tizniti4, Afaf Riffi Amarti2, Nouafal Mellas1, Omar El Mesbahi1 1Department of Medical Oncology, Hassan II University Hospital, Fez, Morocco; *Corresponding Author: [email protected] 2Department of Pathology, Hassan II University Hospital, Fez, Morocco 3Department of General Surgery, Hassan II University Hospital, Fez, Morocco 4Department of Radiology, Hassan II University Hospital, Fez, Morocco Received 22 December 2011; revised 18 January 2012; accepted 6 February 2012 ABSTRACT rent therapeutic options include multiagent chemothe- rapy and aggressive surgical debulking and radiotherapy Desmoplastic small round cell tumor (DSRCT) is [4,5]. The addition of hyperthermic intra-peritoneal che- a rare and highly aggressive variety of sarcoma motherapy in the multimodal approach has been reported arising typically from abdominal or pelvic peri- in very few cases but no effect on survival has been clearly toneum. Diagnosis and treatment approaches of demonstrated [6]. this entity are complex and require a skilled, ex- The prognosis of this disease is poor with a Median perienced, multidisciplinary team. Authors re- survival of 17 months approximately [7]. port their experience with a case of an intra-ab- We report a case of an intra-abdominal DSRCT in a 37 dominal DSRCT arising in a 37-year-old young -year-old young man who was treated with combination man in order to discuss the clinico-pathological chemotherapy and surgery. and radiological behavior of this tumor and un- derline diagnostic and therapeutic difficulties. 2. CASE PRESENTATION Keywords: Desmoplastic Small Round Cell Tumor; A 37-year-old man presented with a 3 months history Abdomen; Histology; Chemotherapy; Surgery; of abdominal distention with discomfort, constipation Outcome and 20 Kg weight loss. His past medical history included pleural tuberculosis treated 20 years ago. Physical ex- amination revealed an 18 cm sized large abdominal mass 1. INTRODUCTION arising from the pelvis. Computed tomography (CT) of Desmoplastic small round cell tumor (DSRCT) is a the abdomen objectified two intraperitoneal masses mea- rare and highly aggressive sarcoma occurring mainly in suring 195 mm and 44 mm long axis respectively in inti- children and young adults [1]. The first case was des- mate contact with the intraperitoneal organs with pelvic cribed in 1989 by Gerald and Rosai [2]. This tumor has ascites and retroperitoneal lymph nodes; These masses predilection for involvement of the peritoneum. A speci- were not confined to any organ-based primary site (Fig- fic chromosomal translocation t (11; 22) (p13; q12) in- ure 1). volving two genes: EWS which is characteristic of Ewing’s CT-guided biopsy of the mass has been performed illu- sarcoma and WT1 which is a Wilms tumor gene, has been strating morphological features of DSRCT, and the diag- documented in DSRCT [3]. nosis was confirmed by immunohistochemistry which re- This malignancy puts diagnostic and therapeutic prob- vealed positivity for cytokeratin and desmin antigen lems; indeed, diagnosis can be suspected by radiological (Figures 2 and 3). and histological features but it is asserted only by im- Induction chemotherapy regimen based on anthracy- muno-histochimic and cytogenetic study because of the clin (60 mg/m2/cycle) and ifosfamide (9 g/m2/cycle) was big number of differential diagnoses and the anatomopa- received. The evaluation after 3 cycles of chemotherapy thologic polymorphism [2,3]. was considered to be in favor of a stable disease by the No curative treatment has been yet documented; cur- RECIST criteria, we stopped chemotherapy due to poor Copyright © 2012 SciRes. OPEN ACCESS 208 H. Benhammane et al. / Health 4 (2012) 207-211 (a) (a) (b) (b) Figure 1. abdomino-pelvic CT showing heterogeneous masses arising from the pelvis measuring 195 mm (a) Figure 3. Immunohistochemical analysis showing and 44 mm (b) long axis, with multiples lymph nodes strong immunoreactivity for epithelial marker -cy- and in intimate contact with intraperitoneal organs. tokeratin antigen- (a) and positivity for muscular marker -desmin antigen- (b). surgery demonstrated: Disappearance of two large intra peritoneal masses with significant reduction in size and number of lymph nodes. Currently, the patient is still alive with a follow up of 18 months from initial diagnosis. 3. DISCUSSION Desmoplastic round cell tumor (DRCT) is a rare vari- ety of sarcoma and a distinct clinico-pathological entity that has been described recently in 1989 [2]. The inci- dence of DRSCT is difficult to estimate because of its rarity; no more than 300 cases have been reported in the literature. This aggressive malignancy is found predomi- Figure 2. Cellular proliferation of uniform small round cells surrounded by a prominent desmoplastic stroma nantly in pediatric age group and young adults with a sex (HES × 100). ratio (male to female) of 3:1. The age ranged from 10 to 16 years in 73% cases [8]. tolerance and we planned a palliative surgical debulking The cell of origin is not yet known. However, it is which included the excision of both masses with subtotal postulated to originate from the serosal lining because it omentectomy, rectosigmoidectomy and terminal colo- is frequently found in the mesothelial-lined surface [9]. stomy. Histological examination confirmed diagnosis of But an epithelial, neurogenic and blastematic origin is DSRCT with no therapeutic effect. CT evaluation after also discussed [10]. Copyright © 2012 SciRes. OPEN ACCESS H. Benhammane et al. / Health 4 (2012) 207-211 209 DSRCT typically arises from abdominal or pelvic peri- Therapeutic options of DSRCT of the abdomen are not toneum. Very rarely, DSRCT can arise from head and neck, well documented due to its rarity. Regarding the ag- base of skull, paratesticular region, ovary, brain and tho- gressiveness of the disease, treatment is based on multi- racic viscera [1]. modal therapy including an extensive surgery when it is Diagnosis of intra-abdominal DSRCT is difficult and feasible, systemic chemotherapy with or without abdo- it is usually obtained at an advanced stage. It may be first minal radiotherapy [12]. It has been reported that the suggested by the imaging findings which are useful also combination of three modalities has shown best results for staging and for a guided biopsy. However, only his- leading to an overall response rate of 39% and a 3-year tological analysis with an immunohistochemic and cyto- survival rate of approximately 50% [1,12] as compared genetic study led to the confirmation of diagnosis [11, to each modality used separately. However, the impact of 12]. In fact, the clinical manifestations are usually non- cytoreductive surgery on survival remains unclear. Some specific; typically, the tumor produces few symptoms authors suggest an improvement in survival with surgery until it is large enough to compress or invade surround- with a median survival of 34 months compared to 14 ing structures. Symptoms include abdominal discomfort/ months for operable and inoperable tumors, respectively distension, abdominal pain, weight loss, or change in [1]. Similarly, Lal in its cohort has reported a significant bowel habits. Radiologically, the most characteristic im- impact of surgery on overall survival with 3 year survival aging feature of DRSCT of the abdomen is single or of 58% as compared to 0% in no resectable disease [15], multiple, lobulated peritoneal soft tissue masses without the benefit is greater after complete resection but fre- an apparent organ of origin. These abnormalities may be quently this act is not feasible due to the extensive in- associated with ascites, lymph nodes, diffuse nodular volvement as the case of our patient. In contrast, other peritoneal thickening or with distant metastasis. The lier, authors have concluded that surgery has no significant lung and bone marrow are common sites of metastases [1, benefit on survival [16,17]. 11]. There are only a few reports on the role of chemothe- In some cases Imaging features are not pathognomonic rapy in DSRCT. Multiagent chemotherapy leads to ap- and can be difficult to interpret due to several differential proximately 40% tumor response [1,12]. The cytotoxic diagnosis that overlap with the appearance of DSRCT agents that seem to be effective in DSRCT are cyclopho- such as Peritoneal carcinomatosis from various primary sphamide, ifosfamide, adriamycin, vincristine, etoposide malignancies, malignant peritoneal mesothelioma, germ and topotecan [12]. Kushner et al. reported some com- cell neoplasm, malignant lymphoma, malignant mela- plete remission with The P6 protocol which has seven noma, and other soft-tissue sarcomas such as malignant courses of chemotherapy (Courses 1, 2, 3 and 6 consisted fibrous histiocytoma. In addition, DSRCT can simulate of HD-CAV, high-dose cyclophosphamide 2100 mg/m2/day the appearance of a benign disease such as desmoid fi- on days 1 and 2, doxorubicin 75 mg/m2/day and vincris- bromatosis, and tumefactive inflammatory processes [11]. tine 2.0 mg/m2/day on days 1, 2 and 3. Courses 4, 5 and The individualization of DSRCT as an antomo-clinical 7 consisted of ifosfamide 1.8 g/m2/day and etoposide 100 entity is based on morphological and especially immuno- mg/m2/day for 5 days) and the progression free survival histochemical criteria: DSRCT are characterized by a could be prolonged [4]. Other authors had highlighted multidirectional and phenotypic expression [12]. Macro- particularly the role of anthracycline based chemothe- scopically, the tumor presents bosselated grey surfaces rapy in some metastatic cases [18]. In fact, chemotherapy with areas of necrosis. Microscopically, tumor manifests may be beneficial in patients with DSRCT; However, as islands of small round cells with hyperchromatic nu- disease recurrence is the rule, often within 6 months [6] clei and scanty cytoplasm surrounded by desmoplastic except two longest survivors reported in the literature stroma [13].
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