Primary Malignant Fibrous Histiocytoma of the

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A malignant fibrous histiocytoma (MFH) is a tumor ... less palpable mass in the right lower abdomen. The pa- .... The anatomic distribution of an intra-abdominal,.

J Korean Radiol Soc 2007;57:549-552

Primary Malignant Fibrous Histiocytoma of the Mesentery: A Case Report 1

Jong Heon Park, M.D., Jae Woo Yeon, M.D., Eun Mee Han, M.D.2, Suk Ki Jang, M.D., Su Min Kang, M.D., In Oak Ahn, M.D.

A malignant fibrous histiocytoma of the mesentery is rare, and multifocal involvement as a primary tumor is very rare. In this report, a case of malignant fibrous histiocytoma of the mesentery presenting with two masses and multiple peritoneal seeding in a 48-year-old man is described. A physical examination revealed a large, firm, and non-tender mass in the right lower abdomen of the patient. Computed tomography of the lesion revealed a partially, indistinctly marginated and heterogeneously enhancing mass with irregular peritumoral strands in the mesentery of the right lower abdomen, while sonograms of the lesion revealed an ill-defined low-echoic mass. The final pathology demonstrated the presence of a storiform-pleomorphic malignant fibrous histiocytoma. Index words : Mesentery

Malignant fibrous histiocytoma Ultrasonography Computed tomography (CT)

A malignant fibrous histiocytoma (MFH) is a tumor that occurs most frequently in the lower and upper extremities and in the retroperitoneum. A primary MFH of the mesentery is rare, and few cases have been reported (1-4). This case report describes the findings of a sonographic image, serial computed tomographic (CT) images, and presents different pathologic diagnoses of such a tumor based on the results of a sono-guided aspiration, a laparoscopic biopsy, and an excisional biopsy. A surgical excisional biopsy of the tumor confirmed the presence of a storiform-pleomorphic MFH in the mesentery. Department of Radiology, Bundang Jesaeng General Hospital Department of Pathology, Bundang Jesaeng General Hospital Received August 13, 2007 ; Accepted October 15, 2007 Address reprint requests to : Jae Woo Yeon, M.D., Department of Radiology, Bundang Jesaeng General Hospital, 255-2, Seohyun-dong, Bundang-gu, Sungnam-si, Gyungki-do 463-774, Korea. Tel. 82-31-779-0051 Fax. 82-31-779-0060 E-mail: [email protected]

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Case Report A 48-year-old man visited our clinic because of a painless palpable mass in the right lower abdomen. The patient detected the mass two weeks prior to the clinic visit. The patient had no history of trauma or any previous surgery. A physical examination of the patient revealed the presence of a large, firm mass in the right lower abdomen. The level of serum carbohydrate antigen 19-9 was elevated, but the other laboratory findings were normal. An initial abdominal CT scan showed an approximate 4.5 cm sized heterogeneously enhancing mass with a partially indistinct margin and irregular peritumoral strands in the mesentery of the right lower abdomen (Fig. 1A). This mass was causing right hydronephrosis. Sonograms showed the presence of an illdefined low-echoic mass. The presumptive diagnoses in-

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Jong Heon Park, et al : Primary Malignant Fibrous Histiocytoma of the Mesentery

A

C

B

D

E F Fig. 1. A 48-year-old man with a palpable mass in the right lower abdomen. A. Computed tomography shows a partially indistinct and heterogeneously enhancing mass with irregular peritumoral strands (white arrows) in the mesentery of the right lower abdomen. B, C. Three months later, follow-up computed tomography shows an increased size of the known mass (white arrows) in the mesentery of the right lower abdomen and another mass (black arrows) with a similar nature in the mesentery of the upper abdomen. D. A microphotograph shows mononuclear histiocyte-like cells and multinucleated giant cells (H & E staining, × 200). E. Immunohistochemical staining for smooth muscle actin shows a positive reaction in the histiocyte-like cells (smooth muscle actin, × 200). F. Microphotographs show irregular peritumoral strands including infiltrating tumor cells and desmoplastic reaction in the mesentery (H & E staining, × 200).

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J Korean Radiol Soc 2007;57:549-552

cluded a variety of tumor or tumor-like conditions of the mesentery causing a desmoplastic reaction, such as a metastatic tumor, carcinoid tumor and sclerosing mesenteritis. An ultrasound-guided aspiration was performed, and the possibility of a benign spindle cell tumor was suggested. Three days later, a laparoscopic biopsy was performed, and the pathologist suggested the possibility of fibromatosis or a gastrointestinal stromal tumor. A complete excision of the mass was recommended for an accurate diagnosis. An excisional biopsy was scheduled, but the biopsy was delayed. Two months later, the abdominal pain and distension became aggravated. A follow-up abdominal CT scan showed an increase in the size of the known mass in the mesentery of the right lower abdomen, and demonstrated the appearance of a new similar mass in the mesentery of the upper abdomen. In retrospect, the initial CT images revealed the presence of multiple mesenteric nodules (Fig. 1B, C). The multiple small, enhancing nodules in the mesentery and the omentum had grown within the two-month period. An extended right hemicolectomy with jejunal resection was performed for palliative treatment. Macroscopically, the surgical specimen contained two ill-defined serosal-intramuscular masses in the mesentery of the jejunum and terminal ileum. Neither necrosis nor hemorrhage was present in the gross specimen. The remaining serosa showed multiple mesenteric nodules, measuring from 0.5 cm to 1.5 cm in the longest dimension. A histopathological examination revealed some areas of a storiform pattern, high cellularity, and marked nuclear pleomorphism with multinucleated giant cells in the tumors of the mesentery (Fig. 1D). Immunohistochemical staining was positive for expression of smooth muscle actin and negative for expression of CD34, C-kit, S-100 protein, desmin, and cytokeratin. These findings were suggestive of a storiform-pleomorphic malignant histiocytoma (Fig. 1E). Five months have elapsed since the surgery, and the patient is doing relatively well without any evident tumor recurrence or distant metastasis. Discussion Primary tumors arising in the mesentery are rare, but the mesentery is a frequent route for the spread of a malignant neoplasm through the peritoneal cavity and between the peritoneal space and the retroperitoneum (5). Most primary lesions are mesenchymal in origin, and most are histologically benign. Malignant mesenchymal

lesions include liposarcomas, leiomyosarcomas, malignant schwannomas, synovial sarcomas, fibrosarcomas and MFHs. Unfortunately, with the exception of a minority of lesions, such as lipomas, hemangiomas and subacute hematomas, the radiological appearance of most soft-tissue masses remains nonspecific (1). An MFH in the abdominal cavity represents 16% of all MFHs, and most of these lesions are located in the retroperitoneum. If secondary involvement of the abdominal cavity can be ruled out, the presence of an intra-abdominal, extra-retroperitoneal MFH could be considered even though multifocal involvement is very rare. The anatomic distribution of an intra-abdominal, extra-retroperitoneal MFH is as follows: the stomach, the small intestine, the colon, the appendix, the mesentery, the liver, and the spleen (1-3). The pathological diagnosis of MFH can be inaccurate if the whole mass cannot be excised as it is composed of variably proportioned histiocytic and fibrous elements. The complete excision of the mass is therefore recommended for an accurate diagnosis. In general, on a CT scan, MFH appears as a relatively well-defined and homogenous mass, or a mass with internal low attenuation due to necrosis or hemorrhage. The frequency of internal necrosis or hemorrhage increases in proportion to the mass size (6). Lee et al. reported 13 masses in the abdomen out of seven cases that were pathologically confirmed to be MFHs; eight of the lesions (62%) had a peritumoral-vascular-appearing structure. Previous reports had not mentioned this finding (4), and Lee and colleagues first reported this finding but there was no pathological confirmation of the presence of a peritumoral-vascular-appearing structure. For MFHs, sonography demonstrates the presence of three patterns, including a hypoechoic pattern (the most frequent), a mixed pattern with an extensive necrotic area, and a predominantly anechoic pattern with thick septa (6). On a CT scan, the MFH appeared as an irregularly margined, heterogeneously enhancing mass with irregular peritumoral strands in the mesentery, and as an illdefined low-echoic mass on the ultrasonogram. The irregular peritumoral strands in this case are similar to the peritumoral-vascular-appearing structure that Lee and colleagues have reported. When these findings were correlated with the pathological findings, they mainly a desmoplastic reaction that included infiltrating tumor cells (Fig. 1B, F). The differential diagnoses of soft-tissue masses with ir-

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Jong Heon Park, et al : Primary Malignant Fibrous Histiocytoma of the Mesentery

regular peritumoral strands in the mesentery include sclerosing mesenteritis, mesenteric edema or hemorrhage, mesenteric inflammation secondary to pancreatitis, fibrofatty mesenteric proliferation related to Crohn’s disease, primary mesenteric neoplasms (e.g., a desmoid or carcinoid tumor), peritoneal mesotheliomas, and metastatic neoplasms (5, 7). Although rare, a MFH should be included in these differential diagnoses. In general, an extra-retroperitoneal abdominal MFH recurs in 37-51% of all cases and metastases occur in 42% of all cases, essentially in the lungs and lymph nodes. The overall two-year and five-year survival rates for patients afflicted with MFH have been reported to be about 60% and 47%, respectively (2). The prognosis of an MFH is unclear, but most of the reports suggested that it is poor. One group of investigators reported that postoperative chemotherapy increased the survival rate, but another group of investigators has reported that postoperative chemotherapy did not achieve curative results. There is a report stating that the tumor was not radiosensitive. However, it is clear that complete surgical excision in the early phase is essential to improve the prognosis, although the role of adjuvant chemotherapy after surgery is still unclear (8-10). In the current case, surgical resection was delayed as the ultrasound-guided aspiration and laparoscopic biopsy suggested that the tumor was benign. Unfortunately, this outcome might have influenced the prognosis of the patient. In conclusion, if CT scan images reveal a large soft-tissue mass with irregular peritumoral strands in the mesentery, especially in a middle-aged adult, an MFH

must be included in the differential diagnoses, along with other benign or malignant disorders. Furthermore, for an accurate diagnosis of an MFH, the whole mass must be excised. References 1. Kransdorf MJ. Malignant soft-tissue tumors in a large referral population: distribution of diagnoses by age, sex, and location. AJR Am J Roentgenol 1995;164:129-134 2. Bruneton JN, Drouillard J, Roqopoulos A, Laurent F, Normand F, Balu-Maestro C, et al. Extraretroperitoneal abdominal malignant fibrous histiocytoma. Gastrointest Radiol 1988;13:299-305 3. Ros PR, Viamonte M Jr, Rywlin AM. Malignant fibrous histiocytoma: mesenchymal tumor of ubiquitous origin. AJR Am J Roentgenol 1984;142:753-759 4. Lee KH, Lee HG, Lee JM, Lee HJ, Ha HK, Baik JH, et al. Radiological findings of primary abdominal malignant fibrous histiocytoma: emphasis on CT findings. J Korean Radiol Soc 1996;35:559-564 5. Sheth S, Horton KM, Garland MR, Fishman EK. Meseneteric Neoplasms: CT appearances of primary and secondary tumors and differential diagnosis. Radiographics 2003;23:457-473 6. Goldman SM, Hartman DS, Weiss SW. The varied radiographic manifestations of retroperitoneal malignant fibrous histiocytoma revealed through 27 cases. J Urol 1986;1352:33-38 7. Horton KM, Lawler LP, Fishman EK. CT findings in sclerosing mesenteritis (panniculitis): spectrum of disease. Radiographics 2003;23:1561-1567 8. Levinson MM, Tsang D. Multicentric malignant fibrous fibrous histiocytomas of the colon. Reporto of a case and review of the subject. Dis Colon Rectum 1982;25:327-331 9. Ekfors TO, Rantakokko V. An analysis of 38 malignant fibrous histiocytomas in the extremities. Acta Pathol Microbiol Scand(A) 1978;86:25-35 10. King RM, Pairolero PC, Trastek VF, Piehler JM, Payne WS, Bernatz PE. Primary chest wall tumors: factors affecting survival. Ann Thorac Surg 1986;41:597-601

대한영상의학회지 2007;57:549-552

장간막의 원발성 악성 섬유성조직구종: 증례 보고1 분당제생병원 영상의학과 분당제생병원 진단병리과

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박종헌・연재우・한은미2・장석기・강수민・안인옥 장간막에 생기는 악성 섬유성조직구종은 드문 원발성 종양이며 다발성으로 생기는 경우는 매우 드물다. 저자들 은 최근 발생한 우하복부에 만져지는 종괴를 주소로 내원한 48세 남자 환자에서 장간막에 발생한 두개의 커다란 종괴와 복막 암종증이 동반된 1예를 경험하였기에 초음파, 컴퓨터단층촬영 및 조직 병리소견을 함께 보고하고자 한 다. 이 종괴는 초음파에서 경계가 불분명한 저에코로 보였고 컴퓨터단층촬영에서는 부분적으로 경계가 불분명한 불 균한 조영증강을 보였으며 주변으로 불규칙한 선상 구조물들이 있었다. 최종 병리 진단은 장간막에서 발생한 다형 성 악성 섬유성조직구종이었다. ─ 552 ─

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