[What is your diagnosis? Answer: unclassifiable renal carcinoma--renal carcinoma with spindle cell and cuboidal components].
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Biomedical subjects
Publications and source records attributed to O Hes.
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Cell cycle progression is governed by cyclin dependent kinases (CDK) that are activated by cyclin binding and inhibited by CDK inhibitors. Protein p27Kip1 functions as a CDK inhibitor, which controls the progression from G1 to S phase. Further, p27Kip1 may have a positive regulative influence. In nonneoplastic tissues and in the majority of tumors investigated so far, the immunohistochemical positivity of p27Kip1 showed an inversely proportional relationship to the proliferation index. Among B-cell non-Hodgkin lymphomas, the exceptions to this rule are represented by mantle cell lymphoma, hairy cell leukemia, and the immunoblastic Epstein-Barr virus latent membrane antigen positive diffuse large B-cell lymphoma in AIDS patients. The loss of p27Kip1 expression is a negative prognostic factor in numerous tumors, including the majority of B-cell lymphomas.
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AIMS: We present the largest series of an unclassified subtype of renal cell carcinoma, which seems to be a distinct morphological entity and which is sometimes designated as spindle and cuboidal renal cell carcinoma. METHODS AND RESULTS: Eleven cases of spindle and cuboidal renal cell carcinoma were found among 7000 primary renal cell tumours in Pilsen's routine and consultation files. The patients were five men and six women. They ranged in age from 22 to 65 years (mean 56.8). Microscopically, the tumours were composed of two main populations of cells. First, the preponderant type of cells was formed by flattened, spindle cells with sparse cytoplasm. The second cell type was a small cuboidal cell with clear to light eosinophilic cytoplasm. Spindle-shaped cells were arranged in a fascicular pattern often reminiscent of low-grade smooth muscle tumours. Solid areas of spindle cells were also present. Small cuboidal cells formed sparse tubular structures lined by a row of single cells. In addition to all previous published cases of spindle and cuboidal renal cell carcinoma we observed an association of nephrolithiasis in our cases. It was seen in 3/11 of our patients. A previously unreported feature is the occurrence of a conventional renal cell carcinoma component in one of our cases. Seven of our patients are currently well without signs of recurrence or metastasis, one had metastasis in a regional lymph node at the time of nephrectomy, one died of an unrelated condition, and two were lost to follow-up. CONCLUSIONS: We present 11 cases of spindle and cuboidal renal cell carcinoma, which is believed to be a distinctive morphological entity. Our cases were histologically, immunohistochemically and ultrastructurally similar to the previously reported cases of spindle and cuboidal renal cell carcinoma. In contrast to all previously reported cases of spindle and cuboidal renal cell carcinoma, we observed an association with nephrolithiasis in three of our cases; moreover, one of our tumours had a conventional renal cell carcinoma component and another revealed a metastatic focus in a regional lymph node. None of our patients died of the disease. This study confirms that spindle and cuboidal renal cell carcinoma has a low malignant potential.
Herein we describe 7 cases of posttransplantation lymphoproliferative disease (PTLD), 5 in men and 2 in women (aged from 25 to 62 years), occurring from 4 months to 12 years (mean, 7 years) after transplantation. Our patients were recipients of kidney, kidney and pancreas, heart, and autologous peripheral haematopoetic stem cells. Four cases were diagnosed as monomorphic and three as polymorphic type of PTLD according to the WHO classification. Monoclonal immunoglobuline heavy chain gene rearrangement was detected in two monomorphic lesions and one polymorphic lesion by polymerase chain reaction (PCR). In the two cases of polymorphic and the one case of monomorphic PTLD, the presence of EBV was visualised by immunohistochemical staining of some transformed lymphoid cells for latent membrane protein (LMP) of EBV. The presence of type A EBV was demonstrated by PCR. The patients were treated by reduction or discontinuation of immunosuppression and by chemotherapy. In 2 cases, a part of the organ affected by lymphoma (sigmoid colon and pancreas) was surgically resected. Four patients died of causes related to PTLD (2 to 15 months after the diagnosis), mainly of infectious complications. Two other patients who achieved remission died of unrelated causes. Only the youngest man is alive and in the complete remission 10 months after the diagnosis of PTLD.
Eleven cases of metanephric adenoma are reported. The tumors were selected out of 6500 tumorous and pseudotumorous lesions of the kidney in our registry. Female to male ratio was 1:1.2. The average age of the patients was 48.3 years, with a range of 13-79 years. The mean size of the tumors was 7.2 cm. The tumors were spherical in shape, whitish to yellowish in colour. Histologically, they were arranged in a mainly tubular pattern with short pseudopapillae. The tumorous cells were deeply eosinophilic to basophilic with predominantly round nuclei. Psammomatous bodies were numerous. Immunohistochemically, they reacted positively with antibodies against cytokeratins, vimentin, and WT1. Ultrastructurally, the cytoplasm contained mitochondria, RER, and ribosomes. A collagenous spherulosis, identical with those in salivary gland and mammary tumors, was revealed in one case. The spherules were located mainly inside tubular structures. Ultrastructurally, they were composed of basement membrane-like material, which was surrounded by epithelial cells. Follow-up all of our patients was negative (if known) for 10 months to 4 years.
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The authors present their initial experience with transplantations of the kidneys from a donor with a non-beating heart (NHBD). This programme was developed because of the permanently declining number of cadaverous kidney donors with a beating heart. Since January 2002 the authors collected a total of three kidneys from NHBD 3 kidneys of which were used for transplantation. The first two started to function immediately, the third one restored its function three weeks after transplantation. The viability and suitability of use of kidneys from NHBD is evaluated with regard to vascular resistance and flow of the perfusion fluid through the kidneys when using a perfusion pump and by means of totalglutathione-S-transferase (tGST) assessed at intervals in the perfusion fluid. The values of the above parameters are limiting between the 6th and 8th hour after the onset of instrumental perfusion of the collected kidneys when the perfusion values should be within the range of 30-50 ml/min/100 g and the vascular resistance should be < 0.7 mmHg and tGST < 100 U/l. For immunosuppression the authors use during the fist days after transplantation basiliximab, rapamycin and corticoids, later in case of satisfactory function of the graft they add cyclosporin A. The initial results of renal transplantation from NHBD are promising and the programme will be developed further.
Kidney tumors represent a wide scale of histological observations. However, only angiomyolipoma can be recognised preoperatively from results of the graphical examination. Other types can be recognised only on the bases of histological examination. Completely benign tumor is oncocytoma (it represents about 5% of all kidney tumors). Angiomyolipoma (2%) is also benign, though some case reports describing its malign transformation has been published. Angiomyolipoma under 4 cm can be only monitored, the larger tumors should be resected or selectively embolised the arterial blood supply to prevent spontaneous rupture. From the group of benign tumors only cystic nephroma can be diagnosed more often (up to 1%). One of the criteria for diagnosing the renal cortical adenoma is its size under 5 mm. That is why any adenoma, which could be diagnosed by means of graphical examination and therefore clinically significant does not exist. Most of tumors are malign epithelial tumors--renal carcinomas (RC). The are classified according Heidelberg classification into 5 elementary types: clear cell, papillary, chromophobe, originating form collecting ducts and not classifiable. Clear cell (conventional) renal carcinoma (CRC) comes most often (70 to 80%), its malign potential rise with increased size of tumor and with the gradient. Five-year survival is achieved in 30-50%. Granular form of CRC carcinoma (7% of all CRC) is the equivalent of poorly differentiated PRC and it has an adverse prognosis. In contrary, the cystic form of CRC (about 6%) in benign. Papillary form of RC has the five-year survival in 84%, malignant are only tumors poorly differentiated. These are tumors with extensive necroses, which brings a fragile consistency and they can be distinguished by graphical examination. Chromophobe type of RC (5%) has the five-year survival in 90%. Poor prognosis has its sarcomatoid form, which can originate from any RC, but most frequently it is derived from the chromophobe type. The form originating from collecting ducts is highly infrequent and very malignant with the five-year survival in 20% only. The unclassified form of RC (3-5%) includes tumors not suiting to the criteria of the previous RC. Other primary renal malignant tumors (sarcomas, Wilms' tumor of adults, medullar carcinoma, carcinoid) are very rare. Comparatively frequent are metastases of other tumors (namely that of lung carcinoma) and renal impairment in leukemia, which are complication not often met by urologist.
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Here we report 3 cases of renal oncocytoma with a dominating small cell component arising in adult women. These 3 cases of small cell variant of renal oncocytoma were found among 134 cases of oncocytomas of the kidney in the routine and consultation files of the authors. Two of 3 cases were initially thought to be a malignant tumor by the referring pathologists. In all 3 cases minor areas of the tumors, which were identical to the classical renal oncocytomas were found. The small cell areas were arranged in a characteristic organoid pattern different from all the known types of the renal benign and malignant tumors. We summarize all morphologic, immunohistochemical, and ultrastructural features of this unusual oncocytoma variant with regard to its differential diagnosis. Int J Surg Pathol 9(3):215-222, 2001
From the collection of 2500 cases of renal epithelial tumors in our files, 102 renal oncocytomas were analyzed for size, multifocality and a morphologic spectrum of the growth pattern. The size of the tumors ranged from 1.5 to 13 cm in diameter, with a mean of 6.3 cm. Three cases were multifocal, four cases were combined with another primary renal tumor (1x angiomyolipoma, 1x conventional renal carcinoma, 2x papillary renal cell carcinoma). A central fibrosis or a scar was noted in 13 cases, and there was a gross area of hemorrhage in 11 cases. In 4 cases extensive necroses were recognized. Histologically, an alveolar pattern was noted in 70 cases. A tubular pattern was revealed in 31 cases and an unusual tubopapillar ("glomeruloid") pattern was noted in one case. Foci of atypical nuclei were identified in 58 cases. In 4 oncocytomas broad areas of clearance of the oncocytes were found. Psammoma bodies were recognized in 9 tumors and foci of ossification were present in 4 cases. Intracellular and extracellular hyaline globules were noted in two cases. Renal oncocytoma has a variable morphologic spectrum, and its diagnosis should be based on an analysis of structural and cytologic features. Differential diagnosis of renal oncocytomas with various tumors of the kidney which contain granular cytoplasm is discussed. These tumors with granular cytoplasm include conventional renal cell carcinomas, chromophobe cell carcinomas, and rare examples of papillary renal carcinomas.
In physiological instances, the prostatic acini may be intermingled with striated muscle fibers in the apex and in the anterolateral subcapsular areas of the prostate. We report an unusual lesion composed of benign prostatic glands, which were scattered between skeletal muscle fibers beneath the bladder submucosa. A 55-year-old man underwent four transurethral resections and radiation therapy for a urothelial carcinoma of the urinary bladder. Subsequently a cystectomy was performed. In the resection specimen, the prostate specific antigen-positive glands were found among the skeletal muscle bundles close to the bladder submucosa. They were seen as small groups of haphazardly scattered or isolated glands mimicking an infiltrative pattern of a prostatic adenocarcinoma. Prominent nucleoli in some glands further contributed to the worrisome appearance of the lesion. However, the high molecular weight cytokeratin 34 beta E12 exhibited basal cell layers of the glands, and the subsequent course of disease confirmed the benign nature of the lesion. The juxtaposition of the prostatic acini with the skeletal muscle out of the apex and the anterolateral subcapsular areas of the prostate should be included in the differential diagnosis of prostatic carcinoma in urinary bladder and prostate biopsies.
We describe a unique benign tumor of the kidney in a 93-year-old man. Microscopically the tumor was composed of an intimate intermixture of epithelial component and smooth muscle tissue, which formed the stroma of the tumor. In some parts the epithelial cells in adenomatous tubular formations were endowed with clear snouts. These clear snouts had a blister-like quality and grew on the secretory cells lining the tubules. No atypias, mitoses, or pleomorphism were present in the tumor. The muscular component consisted of poorly cellular, HMB-45-negative, leiomyomatous bundles, which greatly differed from that of angiomyolipoma. It encircled the whole tumor and intimately intermingled with the epithelial component. These leiomyomatous bands formed focally abortive vessels, which had incomplete and irregular walls and lacked an elastic layer. Even more interesting was a peculiar vascularization of the tumor. All epithelial tubular structures of the tumor revealed an intimate association with small capillaries. A fine labyrinth of the capillaries rimmed the rows of the epithelial cells. To the best of our knowledge this tumor differs from all previously described.
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In the Czech Republic in recent years a steady decline of renal transplantations was recorded due to shortage of organ donors. The authors present initial experience with the collection of organs from dead non heart beating donors (NHBD). In the course of less than two years this programme was introduced within the framework of a clinical experiment (grant IGA MofH CR ND/4781-3) at the Surgical Clinic, Faculty Hospital in Plzen. A total of 20 kidneys were collected from NHBD and another 12 kidneys were not collected, most frequently because of difficulties in communication and organization. The donors were grouped according to the Maastricht classification. The mean age of donors was 43.3 years, the mean period of warm ischaemia was 22 minutes and the serum creatinine level was 145 mumol/l. Of 20 collected kidneys a normal histological appearance was recorded in 14, the remainder had different degrees of damage due to inadequate rinsing. Based on hitherto assembled experience the authors emphasize perfect team collaboration in collection from NHBD and a precise technique of collection. The next stage of the clinical study will be the preparation proper for clinical transplantation of kidneys from NHBD.