Primary retroperitoneal liposarcoma

European Journal of Obstetrics & Gynecology and Reproductive Biology 109 (2003) 228–230

Case report

Primary retroperitoneal liposarcoma Mehmet A. Osmanag˘aog˘lua,*, Hasan Bozkayaa, ¨ zerena, U ¨ mit C¸obanog˘lub Mehmet O a

Department of Obstetrics and Gynecology, Medicine School of Black Sea Technical University, 61080 Trabzon, Turkey b Department of Pathology, Medicine School of Black Sea Technical University, 61080 Trabzon, Turkey Received 29 September 2001; received in revised form 8 August 2002; accepted 14 November 2002

Abstract A retroperitoneal liposarcoma that marginally involved the right kidney and ovary was presented clinically as an adnexial mass in a 61-yearold woman. At laparatomy a large retroperitoneal mass in addition to the right kidney and ovary was removed totally. A well-differentiated retroperitoneal liposarcoma was detected in histological speciment. Adjuvant irradiation was given. The 5-year survival rate of retroperitoneal liposarcoma is low. Curative resection remains the main treatment for primary and recurrent liposarcomas. # 2002 Published by Elsevier Science Ireland Ltd. Keywords: Retroperitoneal liposarcoma

1. Introduction Liposarcoma is a rare mesenchymal tumor that occurs most commonly in the soft tissues of the extremities, but other sites such as the retroperitoneum can be involved. Retroperitoneal liposarcomas grow slowly in the very expandable retroperitoneal space in the deeply hidden and clinically silent retroperitoneum [1]. It can extend retroperitoneally into the female pelvis representing a potential pitfall for gynecologist, who can misdiagnose them as adnexal masses [2–4]. Because of the rarity of this lesion it is difficult for the surgeon to accumulate a large treatment experience. Its prognosis is poor due to tumor relapse and only complete surgical removal produces a ‘‘cure’’ [1,5]. Therefore, our aim was to understand the anatomic extension of these tumors to better plan surgical treatment and to determine the outcome of these patients.

2. Case report A 61-year-old woman presented with an abdominal mass. Her early symptoms, including vague digestive disturbances and increasing abdominal distension. Her medical history was unremarkable. On pelvic examination, a mass extending ¨ niversitesi, Corresponding author. Present address: Karadeniz Teknik U Farabi Hastanesi, Kadin Hastaliklari ve Dog˘um ABD, 61080, Trabzon, Turkey. Tel.: þ90-462-377-54-19; fax: þ90-462-325-05-18. *

from the right adnexa to the epigastric region has been found. Ultrasonography and CT scan led to a preoperative diagnosis of ovarian tumor. On IVP the left kidney was normal, the right kidney was functional but there were caliceal distortion without any dilatation. At laparotomy, through a longitudinal, supraumbilicopubic midline incision, the pelvic organs were found displaced to the left by a large, well-circumscribed retroperitoneal mass, 30
15 cm in dimension, that marginally involved the right kidney and ovary. The right ovary was cystic and 3
4 cm diameter and also, covered partially by the omentum. The right adnexa and the adherent omentum were removed totally and the multiple biopsies were taken from the mass. Intraoperatively, frozen section diagnosis of the biopsies was atypical but benign for the right adnexial piece. The tumor mass was excised by blunt dissection, extended upward, taking care that it does not jeopardize the vital organs such as great vessels. The right kidney closed the mass and moved together, so it was removed with possible microscopic involvement. The uterus and the left adnexa was also removed because of myomatosis uterus. Her postoperative course was uneventful. Histologically, it consisted in tumor, well separated from peripheral lipomatous tissue with a thin fibrous capsule. Neoplastic cells showed prominent spindling with variably sized hyperchromatic nuclei. Scattered multivacuolated lipoblasts were seen in myxoid stroma. Immunohistochemically, lipoblasts showed reactivity with S-100. Focal

0301-2115/02/$ – see front matter # 2002 Published by Elsevier Science Ireland Ltd. doi:10.1016/S0301-2115(02)00484-0

M.A. Osmanag˘aog˘lu et al. / European Journal of Obstetrics & Gynecology and Reproductive Biology 109 (2003) 228–230

vimentin positivity was noticed in spindle cells. Tumor consisted in large necrotic areas, perivascular chronic inflammatory infiltration and chondroid bone metaplasia. In histological sections of kidney and ureter, plasma cells with rich inflammatory infiltration was noticed in renal paranchyme and beneath the ureteral epitelium. No tumor extension or metastasis was found in uterus, ovaries, tubes and right kidney. The histology of the right ovary showed a benign serous cyst. The result of DNA analyses reported that the tumor was aneuploidy. According to mitotic activity and pleomorphism, the final histological diagnosis of speciment was well-differentiated liposarcoma. The patient received radiotherapy consisting of 30 Gy to the whole abdomen with 26 Gy boost to the involved area by using 10 MV linear accelerator. The patient is yet under follow-up 12 months with regular clinical examinations and CT scans every 3 months. There was no findings about recurrence.

3. Discussion Retroperitoneal liposarcoma produces nonspecific symptoms and is often extensive when diagnosed [3,6]. The presenting symptoms depend on the structure or organ that is displaced, stretched, or compressed by the tumor. These tumors may appear at any age but the majority occur in the 40–60 years age group [1]. Liposarcoma and leiomyosarcoma are the most frequently diagnosed sarcomas in the retroperitoneal space [5,6]. Computerized tomography or MRI were useful in evaluating the soft tissue tumors and the involvement of the other organs. They are also valuable in the follow-up and are useful in detecting recurrence early. Preoperative IVP should be routine in patients with retroperitoneal sarcomas, because nephrectomy is required in 50–70% of complete resections. lntraoperative open biopsy is required for a correct diagnosis and to allow adequate surgery [5]. Complete surgical excision as the most effective primary therapeutic approach and prerequisite for potential longterm survival and local control in patients with retroperitoneal liposarcoma, because all local recurrences developed in or adjacent to the original tumor site [7], and preserved organs such as kidney were found to be highly related with local recurrence [8]. Therefore, aggressive surgical management is strongly recommended for primary or recurrent retroperitoneal liposarcomas [9] as was performed in our case that the kidney was removed. Liposarcoma is a rare but a highly heterogeneous disease and its outcome is determined to be of significant degree by many factors such as histological subtype, tumor location, age older than 50 years, tumor size greater than 10 cm, positive microscopic margins, and locally recurrent presentation [10]. Clinical behavior is associated with histology. The most significant parameters in grading liposarcoma were tumor differentiation, mitotic count, and extent of

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necrosis. Low-grade lesions show a high incidence of local recurrence but little to no propensity for metastasis, and exhibited good prognosis for long-term survival, in contrast with high-grade or poorly differentiated tumors, which often manifest clinically aggressive behavior with a high incidence of local recurrence and distant metastasis [10]. The presented case had an aneuploid tumor. In contrast, well-differentiated sarcomas may demonstrate DNA aneuploidy. It is suggested that tumor ploidy should be analyzed in every case of retroperitoneal sarcoma for better assessment of prognosis and possible indication for adjuvant therapy. It is reported that, only DNA ploidy was an independent prognostic variable. Relative risk of death was over three times higher for aneuploid than for diploid tumors [11]. On the other hand, the cellular makers including DNA ploidy, p53 gene and proliferative activity might be less useful in huge liposarcomas in retroperitoneum, because of its heterogenity increased [12]. Postoperative radiotherapy was given in the presented case, because there was a bulky mass (up to 10 cm) preoperatively, and the assessment of microscopic margins remains difficult. Despite adequate surgical excision, death occurred in the majority of these patients because of the high rate of local recurrence which has prompted several investigators to use intraoperative or postoperative radiotherapy [7,10], and the best results were seen after extended resection followed by postoperative radiation therapy [7]. On the other hand, the other trials on neoadjuvant or adjuvant radiation therapy for retroperitoneal sarcoma, failed to prove prognostic value [5,9]. There is no evidence to support the use of adjuvant chemotherapy for retroperitoneal sarcoma [9]. Because of the high rate of local recurrences, on follow-up CT or MRI re-evaluation of the patient every 3–6 months during the first 2 years after operation and every 6 months for 3 years thereafter is essential [5].

4. Comment Local control accomplished by complete surgical resection with and without adjuvant radiation therapy for retroperitoneal liposarcomas should impact strongly on prognosis and adequate follow-up is essential. References [1] Witz M, Shapira Y, Dinbar A. Diagnosis and treatment of primary and recurrent retroperitoneal liposarcoma. J Surg Oncol 1991;47: 41–4. [2] Susini T, Taddei G, Massi D, Massi G. Giant pelvic retroperitoneal liposarcoma. Obstet Gynecol 2000;95:1002–4. [3] Hsiao JC, Yang YC, Lin WC, Lin YN, Wang KG. Primary retroperitoneal liposarcoma mimicking ovarian cancer: a case report. Chung Hua I Hsueh Tsa Chih (Taipei) 1998;61:295–300. [4] Kuppuvelumani P, Rachagan SP, Syed N, Kumar G, Cheah PL. Rare case of huge retroperitoneal liposarcoma presenting as a

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[5] [6]

[7] [8]

M.A. Osmanag˘ aog˘ lu et al. / European Journal of Obstetrics & Gynecology and Reproductive Biology 109 (2003) 228–230 gynaecological problem. Eur J Obstet Gynecol Reprod Biol 1993;48: 220–2. Rossi CR, Nitti D, Foletto M, et al. Management of primary sarcomas of the retroperitoneum. Eur J Surg Oncol 1993;19:355–60. Blanken R, Meijer S, Cuesta MA, Blomjous CEM. Retroperitoneal sarcomas: pre-operative assessment and surgical therapy. The Netherlands J Surg 1991;43:245–8. van Doorn RC, Gallee MPW, Hart AAM, et al. Resectable retroperitoneal soft tissue sarcomas. Cancer 1994;73:637–42. Li YK. Management of perirenal liposarcoma. Zhonghua Zhong Liu Za Zhi 1993;15:65–7.

[9] Malerba M, Doglietto GB, Pacelli F. Primary retroperitoneal soft tissue sarcomas: results of aggressive surgical treatment. World J Surg 1999;23:670–5. [10] Linehan DC, Lewis JJ, Leung D, Brennan MF. Influence of biologic factors and anatomic site in completely resected liposarcoma. J Clin Oncol 2000;18:1637–43. [11] Herman K, Gruchala A, Niezabitowski A, Glinski B, Lackowska B. Prognostic factors in retroperitoneal sarcomas: ploidy of DNA as a predictor of clinical outcome. J Surg Oncol 1999;71:32–5. [12] Sato T, Nishimura G, Nonomura A, Miwa K. Intra-abdominal and retroperitoneal liposarcomas. Int Surg 1999;84:163–7.