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1 中華放射醫誌 Chin J Radiol 004; 9: Intra-abdominal Desmoplastic Small Round Cell Tumor: CT and MR Findings: a case report WEN-YU LEE CHIN-MING JENG CHIH-YU HSU YUNG-CHUAN SUNG SHIH-HUNG HUANG 3 YOUNG-CHEN WANG CHIN-HUE KUNG CHAN-YING WU Department of Radiology, Internal Medicine, Pathology 3, Cathay General Hospital Desmoplastic small round cell tumor is a rare malignant small cell neoplasm which tends to occur in adolescents and young adults. This tumor usually presents as an abdominopelvic mass with intraperitoneal and hepatic metastasis. We report a case presenting as abdominal masses with unilateral pleural seeding. A 5-year-old woman without previous history of major diseases came to our hospital because of shortness of breath for weeks. Chest X-ray revealed massive amount of left pleural effusion. CT and MRI suggested two intraabdominal masses with multiple left pleural seeding and massive left pleural effusion. The definitive diagnosis was made with the aid of echo-guided fine needle biopsy. Key words: CT; Desmoplastic small round cell tumor; Magnetic resonance imaging Reprint requests to: Dr. Chin-Ming Jeng Department of Radiology, Cathay General Hospital. No. 80, Sec. 4, Jen Ai Road, Taipei 06, Taiwan, R.O.C. Desmoplastic small round cell tumor is an aggressive malignant small round cell neoplasm that mainly affected adolescents and young adults. It usually presents as abdominal discomfort with abdominal mass []. The purpose of this paper is to describe a rare case of desmoplastic small round cell tumor presenting as shortness of breath with massive left pleural effusion on CT & MRI. CASE REPORT A 5-year-old girl was quite well until two weeks ago. She was sent to a local hospital due to shortness of breath. Fever was also noted. Massive left pleural effusion was found. A pig-tail catheterization for effusion drainage was undertaken at that time. She was then transferred to our hospital for further treatment. Physical examination at emergency department showed diminution of breath sound at left lower lung field. The cytologic findings in pleural effusion revealed many large pleomorphic hyperchromatic cell with prominent nucleus and small lymphocytes. Plain chest radiographs showed massive left pleural effusion without shifting of mediastinum. Trachea was at the midline (Fig. ). Chest CT showed nodular thickening of left pleural surface with massive left pleural effusion (Fig.a). Abdomen CT revealed two heterogeneous masses over left upper abdomen (Fig.b). No definite calcification of the masses was found. Neither abdominal lymphadenopathy nor hepatic metastasis was noted. Chest and abdomen MR imagings revealed two masses over left upper abdomen. They were inhomogeneous low signal intensity on T*weighted image (SPGR), intermediate signal intensity on T-weighted image, heterogeneous contrast enhancement on Gd-T- weighted image. Circumferential nodular thickening of left pleura with massive left pleural effusion was revealed (Fig.3a, 3b, 3c, 3d).

00 Intraabdominal desmoplastic small round cell tumor The definite diagnosis was made by echo-guided fine needle biopsy from the left infradiaphragmatic area.

The patient received chemotherapy. She is regularly followed up at OPD. After therapy for 6 months, her general condition is stable.

It is characterized by a reciprocal translocation t(;)(p3;q) associated with the EWS-WT (Ewing sarcoma-wilms tumor ) gene fusion transcript []. It has a male predominance in previous reports [3].

2 00 Intraabdominal desmoplastic small round cell tumor The definite diagnosis was made by echo-guided fine needle biopsy from the left infradiaphragmatic area. Histological examination showed desmoplastic round cell tumor (Fig.4). No pleural biopsy was done. According to histological findings, the pleural lesion was regarded as metastasis. The patient received chemotherapy. She is regularly followed up at OPD. After therapy for 6 months, her general condition is stable. DISCUSSION Desmoplastic small round cell tumor is a rare, aggressive neoplasm that tends to affect adolescents and young adults. It is characterized by a reciprocal translocation t(;)(p3;q) associated with the EWS-WT (Ewing sarcoma-wilms tumor ) gene fusion transcript []. It has a male predominance in previous reports [3]. But a nearly equal sex ratio is reported by Pickhardt et al [4]. The manifestations of desmoplastic small round cell tumor are related to its location. The presentations of intra-abdominal desmoplastic small round cell tumor are usually non-specific such as abdominal or pelvic symptoms and palpable masses. The typical histological findings of desmoplastic small round cell tumor is variable-sized clusters of small, round or spindled cells lying in a desmoplastic stroma []. The characteristic CT presentation of desmoplastic small round cell tumor is single or multiple peritoneal masses without any original organ that are located within the omentum or the mesentery, or adjacent to the bladder [4]. Tumors are often inhomogenous on CT due to focal hemorrhage or necrosis. Lymphadenopathy, punctate calcification of tumor, diffuse peritoneal thickening, hydronephrosis and hepatic metastasis are less common findings [3,4,5,6]. Pleural dissemination is accompanied by disease spreading in one study [3]. In our case, two intraperitoneal masses without other intraperitoneal manifestation were found. Obvious pleural rind with massive pleural effusion was noted. Characteristics MRI findings of desmoplastic small round cell tumor are intermediate signal intensity on T-weighted images, high signal intensity Figure. CXR shows massive left pleural effusion without shifting of the mediastinum. The trachea was at the midline. a Figure. a. Contrast-enhancing CT at the level of hepatic dome shows nodular thickening (white arrowheads) of left pleura with massive left pleural effusion, a pig-tail catheter in place. b. Contrast-enhancing CT at the level of celiac trunk shows two heterogeneous masses (black arrows) over left upper abdomen. b

Intraabdominal desmoplastic small round cell tumor 0 3a 3b 3c Figure 3. a. Axial T-weighted image (TR/TE = 445/40.

3ms, FSPGR/0) shows two heterogeneous co

Axial T-weighted contrastenhancing image shows inhomogeneous contrast enhancement of the nodular thickening of pleura (black arrows) of left lower pleural space. d.

pleural effusion. 3d on T-weighted, inhomogeneous contrast enhancement on gadolinium-enhanced T-weighted images [3,7].

3 Intraabdominal desmoplastic small round cell tumor 0 3a 3b 3c Figure 3. a. Axial T-weighted image (TR/TE = 445/40.5ms, FSE-XL/90) shows two intermediate signal intensity masses (black arrows) over left upper abdomen. b. Axial Gd-T-weighted image (TR/TE=7.8/4.3ms, FSPGR/0) shows two heterogeneous contrast enhancing masses (black arrows) over left upper abdomen. c. Axial T-weighted contrastenhancing image shows inhomogeneous contrast enhancement of the nodular thickening of pleura (black arrows) of left lower pleural space. d. Coronal T-weighted contrast-enhancing image shows heterogeneous contrast enhancement of the left upper abdominal masses (black arrow), nodular thickening of left pleura (white arrows) with left pleural effusion. 3d on T-weighted, inhomogeneous contrast enhancement on gadolinium-enhanced T-weighted images [3,7]. MRI can well depict tumor location and extension by multiplanar images than other modalities. Because no specific features of intra-abdominal desmoplastic small round cell tumor. It must be differentiated with multiple peritoneal masses without obvious original organ. The differential diagnosis includes malignant mesothelioma of peritoneum, non- Hodgkin s lymphoma, malignant gastrointestinal stroma tumor, pseudomyxoma peritonei, peritoneal metastasis, tuberculosis. The malignant mesothelioma of peritoneum seems to present as small modules lining the surface of parietal peritoneum with a dominant mass in one part of abdomen. The non- Hodgkin s lymphoma usually mimics carcinomatosis with mesenteric and retroperitoneal lymphadenopathy. The pseudomyxoma peritonei is low-attenuation masses with scalloping parenchymal organ margins on CT. The peritoneal metastasis comes from ovarian and gastrointestinal primaries with masses and soft tissue infiltration and ascites. The tuberculosis may show low attenuation lymphadenopathy, exudative ascites, mesenteric masses on CT. The treatment of desmoplastic small cell is difficult including chemotherapy, radiotherapy,

0 Intraabdominal desmoplastic small round cell tumor surgical resection. Outcome of the majority of cases are poor despite of aggressive therapy.

4 0 Intraabdominal desmoplastic small round cell tumor surgical resection. Outcome of the majority of cases are poor despite of aggressive therapy. The patients usually die within the first years after diagnosis. However, recent studies of combined modality protocol associated with autologous peripheral blood stem cell transplantation have been tried [8]. Figure 4. Photomicrography of the specimen shows the tumor composed of cohesive epithelial-like nests and trabeculae of small to medium-sized, round hyperchromatic cells, with surrounding desmoplastic stroma (Hematoxylin and eosin stain, x 00). REFERENCE. Gerald WL, Miller HK, Battifora H, et al. Intra-abdominal desmoplastic small round-cell tumor: report of 9 cases of a distinctive types of high-grade polyphenotypic malignancy affecting young individuals. Am J Surg Pathol 99; 5: Lae ME, Roche PC, Jin L, et al. Desmoplastic small round cell tumor: a clinicopathologic, immunohistochemical, and molecular study pf 3 tumors. Am J Surg Pathol 00; 6: Tateishi U, Hasegawa T, Kusmoto M, et al. Desmoplastic small round cell tumor: imaging findings associated with clinicopathologic features. J Comput Assist Tomogra 00; 6: Pickhardt PJ, Fisher AJ, Balfe DM, et al. Desmoplastic small round cell tumor of the abdomen: radiologichistopathologic correlation. Radiology 999; 0: Varma DG, McDaniel K, Ordonez NG, et al. Primary malignant small round cell tumor of the the abdomen: CT findings in five cases. AJR 99; 58: Wang HK, Chen JD, Tiu CM, et al. Intra-abdominal desmoplastic small cell tumor: CT and ultrasound images. Clin J Radiol 999; 4: Outerwater E, Schiebler ML, Brooks JJ. Intraabdominal desmoplastic small round cell tumor: CT and MR findings. J Comput Assist Tomogr 99; 6: Kurre P, Felgenhauer JL, Miser JS, et al. Successful dose-intensive treatment of desmoplastic small round cell tumor in three children. J Pediatr Hematol oncol 000; :

5 Intraabdominal desmoplastic small round cell tumor 03 腹腔內促纖維性小細胞瘤 : 電腦斷層及磁振影像之病例報告李紋瑜鄭慶明徐志育宋詠娟 3 黃世鴻王永成孔慶惠吳昭瑩國泰綜合醫院放射線科內科 3 病理科促纖維性小細胞瘤是一種十分罕見的惡性腫瘤, 好發於青少年及年輕人通常以腹部骨盆腫瘤併腹膜及肝轉移表現我們要報告一例以腹部腫瘤表現併單側肋膜轉移一位 5 歲之女性, 以前沒有疾病史, 由於兩週呼吸急促來本院就診胸部 X 光片發現大量左側肋膜積水, 電腦斷層及磁振影像發現腹腔內腫瘤合併多處左側肋膜轉移及大量肋膜積水確定診斷是靠超音波下細針切片關鍵詞 : 電腦斷層 ; 促纖維性小細胞瘤 ; 磁振影像

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