Przemysław Adamczyk 1, Romuald Butkiewicz 2, Joanna Nussbeutel 2
- 1 Department of Urology, Municipal Hospital in Toruń
2 Department of Pathomorfology, Municipal Hospital in Toruń
horseshoe kidney, malignant fibrous histiocytoma, renal malignant tumors
- Malignant fibrous histiocytoma is very rare type of kidney tumor. It is considered to be "atypical renal tumor", thus classical symptoms like haematuria, pain, temperature is usually not present. Usually first symptoms are pain, and mass in abdomen. The prognosis usually is very poor. Article presents short report of the case with literature review and presents possible ways of following treatment.
Renal tumors classified as malignant fibrous histiocytoma are very rare. To our knowledge only 10 cases of malignant fibrous histiocytoma tumors of the kidney were reported, among them 1 arising from the renal pelvis and 1 involving the renal capsule. All authors described small size tumors arising from renal matrix, but there was no case of this size, and this type of tumor in horseshoe kidney.
A 75-year-old man was admitted to the hospital in May 2003 with a 4-year history of abdominal tumor. The ultrasonography was preformed in 1999 and 2001 and presented a 45 mm in diameter tumor arising from left horseshoe kidney. The patient was not treated in that time, because of his decision of so. During the last three months a large mass was observed, deforming left part of the abdomen, which made the patient start therapy. Other symptoms, including haematuria, pain or temperature were not present. The patient was not suffering from any other major diseases.
Physical examination revealed a solid child head-sized left abdominal mass, confirmed with USG and computerized tomography, which revealed enlargement of the left horseshoe kidney, 200 mm in diameter, deforming abdomen cavity and moving its entire consistence to the right side.
Radical transperitoneal nephrectomy was performed, during which a giant mass (2100 g and 200 mm in diameter) arising from the left part of horseshoe kidney was removed. Frontal part of the tumor was attached to sigmoid colon therefore partial resection of the sigma was performed with cutaneous stoma. The rear part of the mass was attached to the iliopsoas muscle, and it had to be removed with a part of it.
During the removal of the giant mass, spleen vessels with spleen capsule were damaged, therefore splenectomy was done during the procedure.
Postoperatively, stoma was complicated with necrosis of its distal edge. It was pulled up and necrotic part was dissected, during another procedure done 5 days later. The patient complained of left abdominal pain, which moderated in two weeks after the first operation.
Microscopic evaluation (dr J. Nussbeutel) revealed malignant fibrous histiocytoma G-2 infiltrating sigmoid colon and iliopsoas muscle.
Discussion and literature review
Primary renal malignant fibrous histiocytoma is a very rare type of the tumor. Only few cases were reported [2,3,4,5,6,8,9,10,11], and the case of such large tumor was not seen in history of our Department, as well as in any of the reports. In the case of the patient operated in our department, the tumor aroused from horseshoe kidney. In addition to that, tumor was attached to the sigmoid colon and iliopsoas muscle, which complicated operation procedure.
Weiss and Enzinger believe , that this type of tumor as a point of its start has histiocytic and fibroblastic line. Its origin from one line of cell - histocytes which have potential to act as facultative fibroblast is also discussed [12,13].
Frequent incidence of malignant fibrous histiocytoma is in the 7th decade of life, and usually is a disease of the extremities tissue, rather than abdomen. Only in 16% of cases tumor was found in retroperitoneal area . Authors describe very slight symptoms [2,3,4,5,6,8,9,10,11]. Haematuria or temperature is very rarely present, and can be seen only in final stage of the disease. Usually first symptoms are pain (57%) and mass in abdomen (44%) [12,13], which was also observed in the case of the patient operated in our department. Sometimes, patient can develop signs of the increased intraabdominal pressure such as varicocele or hernia . Quite frequent sign of the tumor may be central necrosis, which may create diagnostic problems with renal cysts .
The prognosis usually is poor in this type of tumor. Only in 32% of patients metastases were not observed in less than 2 years. Intraoperatively local metastases and infiltrations of surrounding tissues may be observed in 54% of patients, operated because of retroperitoneal masses diagnosed of malignant fibrous histiocytoma . Correlation between depth of infiltration and prognosis were found. Deeper tumors had a greater likelihood of forming metastases than superficial ones. A muscle infiltration was also bad prognosis factor . Only few positive factors were found among which company of large amounts of chronic inflammatory cells had better prognosis than in overall group.
Weiss and Enzinger divided MFH in 3 groups - predominantly myxoid, myxoid and predominantly cellular. The first group of tumors has better prognosis than others . In our case myxoid, the variant of tumor was found.
Five weeks after the treatment, a large mass in retroperitoneum was observed (USG), with no response to any treatment. Patient died two weeks later.
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