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Journal of Postgraduate Medicine, Vol. 49, No. 3, July-Sept, 2003, pp. 246-248 Case Report Primary Giant Cell Malignant Fibrous Histiocytoma of the Kidney with Staghorn Calculi Chen CH, Lee PS, Han WJ, Shen KH
Division of Urology, Department of Surgery, Chi Mei
Medical Center, Tainan, Taiwan. Code Number: jp03066 ABSTRACT Malignant fibrous histiocytomas (MFH) as primary renal tumours are rare, with less than 50 cases described in the literature. We report a case of primary renal MFH of giant cell type in a 56-year-old man, who presented with bilateral dull flank pain, intermittent gross haematuria and body weight loss (6 kg in 3 months). Intravenous urography, computerized tomography (CT) and magnetic resonance image (MRI) showed right ureteral stones with mild hydronephrosis, and a solid mass at the lower pole of the left kidney associated with staghorn calculi, as well as tumour thrombi in the left renal vein and inferior vena cava. Left radical nephrectomy and evacuation of tumour thrombi from the left renal vein and inferior vena cava were performed. Histopathologic examination revealed malignant fibrous histiocytoma (MFH) of giant cell type. To the best of our knowledge, this is the first report of primary renal MFH associated with staghorn calculi. Keywords: Maligant fibrous histiocytoma, Staghorn calculi, Kidney Malignant fibrous histiocytoma (MFH) is the most common soft tissue sarcoma of adults and was first described by O'Brien and Stout in 1964.[1] Typical locations are the extremities (67-75%) and the retroperitoneum (6-16%).[2] Primary renal MFH is rare with less than 50 cases reported in the literature to date.[2],[3],[4] Primary renal MFH associated with renal staghorn calculi has never been reported before. We present the clinicopathological features of a case of primary renal MFH of giant cell type associated with staghorn calculi. CASE HISTORY A 56-year-old man presented with bilateral dull flank pain, intermittent gross haematuria and body weight loss of more than 6 kg in the past 3 months. Physical examination revealed mild tenderness over both costo-vertebral angles but no palpable flank mass. Laboratory analysis revealed anaemia (Hb 10.9 gm/dL), leukocytosis (22,200/cumm) and borderline azotaemia. Chest X-Ray was unremarkable. Intravenous urography showed a right ureteral stone with mild hydronephrosis and a solid renal mass associated with staghorn calculi at the lower pole of the left kidney. Computerized Tomography (CT) and Magnetic Resonance Imaging (MRI) confirmed the right upper ureteral stone and an 8-cm left renal mass associated with staghorn calculi, and tumour thrombi in the left renal vein and inferior vena cava [Figure-1] & [Figure-2]. The adjacent organs were normal. He underwent ureteroscopy to remove the right upper ureteral stone, followed by left radical nephrectomy and tumour removal from the left renal vein and inferior vena cava. The postoperative course was uneventful. He then received 6 cycles of local irradiation and subsequent chemotherapy with doxorubicin and ifosfamide. He has been followed up for 12 months without evidence of tumour recurrence. The left kidney and perirenal tissue weighed 854 gm. The cut surface revealed a firm tan lobated tumour of the lower pole measuring 8x5x4 cm. [Figure-3]. Staghorn calculi were lodged in the renal pelvis and lower calyces. Histologically, the tumour was extensively necrotic and composed of pleomorphic spindle to polygonal cells arranged in fascicles or whorls. Numerous osteoclast-like multinucleated giant cells and frequent mitotic figures were seen. These giant cells are benign and are not a malignant neoplastic component. Immunohistochemical stains demonstrated the tumour cells were negative for epithelial markers (cytokeratins, AE1/AE3 and CAM5.2) but positive for vimentin and CD68 [Figure-4], consistent with MFH of giant cell type. The tumour invaded beyond the renal capsule and caused tumour thrombo-embolism in the left renal vein and inferior vena cava. No regional lymph node metastasis was noted. DISCUSSION The possibility of a renal tumour can be easily overlooked in a patient presenting with the classic symptoms and signs of renal calculi. However, body weight loss is atypical for uncomplicated calculus disorder and should alert us to consider renal calculi complicated by associated malignancy. Retroperitoneal MFH does not present clinical symptoms distinct from other retroperitoneal neoplasm. As in our case, the presenting symptoms included bilateral flank pain, intermittent gross haematuria and body weight loss. Preoperative image studies cannot distinguish MFH from renal cell carcinoma.[2],[5],[6] The association between this rare tumour and calculi is an occasional finding. Definitive diagnosis of MFH relies on histopathologic examination. Diagnosis of MFH is essentially a diagnosis of exclusion which is rendered when dealing with a malignant sarcoma with marked cellular pleomorphism, exhibiting no specific cell lineage of differentiation except that of a generic fibroblastic (vimentin positivity) and, less importantly for diagnostic purposes, a possible histiocytic (CD68 positivity) one. Radical surgery offers the only chance of cure in all retroperitoneal sarcomas.[7],[8] Despite radical surgery, MFH shows a strong tendency for local recurrence (> 50%), and distant metastases to the lungs and bone.[8] In contrast to angiosarcoma and leiomyosarcomas, MFH appears to be chemotherapy-sensitive, particularly with doxorubicin and ifosfamide-based regimens.[5],[8] Nevertheless, the overall prognosis is unfavourable. Important prognostic parameters include tumour size and histological grade.[6],[7],[8] The relationship between renal calculi and squamous cell carcinoma has been well documented[9] but no association of MFH and renal calculi has been reported. The treatment for giant cell type MFH is not yet standardised. REFERENCES
Copyright 2003 - Journal of Postgraduate Medicine. Online full-text also available at http://www.jpgmonline.com/ The following images related to this document are available:Photo images[jp03066f4.jpg] [jp03066f3.jpg] [jp03066f1.jpg] [jp03066f2.jpg] |
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