Case of primitive neuroectodermal tumor of the kidney

A 28-year-old woman presented with right flank pain. A large, firm, fixed mass was palpable in the right side of the abdomen. Computed tomography revealed a solid mass of the right kidney with extension into the renal vein and inferior vena cava. The patient underwent right radical nephrectomy with en bloc resection of the inferior vena cava containing tumor thrombus and right adrenalectomy. Histologically the tumor consisted of small tumor cells with rosette formation. Immunohistochemical staining was positive for CD99 and NSE. Analysis with polymerase chain reaction (PCR) demonstrated the EWS/FLI1 fusion products resulting from a chromosomal translocation. These findings were consistent with primary renal primitive neuroectodermal tumor (PNET). Two months after surgery, multiple lung, liver and lymph node metastases were found. The patient received 2 cycles of chemotherapy with cisplatin, ifosfamide, etoposide, resulting in a partial remission. She subsequently received 1 cycle chemotherapy with paclitaxel and carboplatin, resulting in no response. The metastatic lung and liver diseases progressed and she died 5 months after diagnosis.     

泌尿系统原发性原始神经外胚层瘤临床病理分析

目的 探讨泌尿系统原发性原始神经外胚层瘤(primitive neuroectodermal tumor,PNET)的临床病理特征、免疫学表型、治疗方法及预后.方法 回顾性分析3例泌尿系统原发性PNET患者资料.3例均为男性,年龄分别为29、32和75岁.2例原发于肾脏,1例原发于膀胱.2例肾肿瘤大小分别为7.7 cm×6.2 cm和12.6 cm×9.4 cm,影像学检查提示肿瘤边界尚清,内部回声欠均匀.膀胱肿瘤大小为10.0 cm×10.0 cm,影像学检查提示膀胱壁不规则增厚,其内密度不均匀.2例肾肿瘤行肿瘤根治术,膀胱肿瘤行血块取出术及肿瘤活检术.结果 光镜下,瘤细胞为形态一致的小圆形或卵圆形,被纤维结缔组织分隔成实性片状或巢状,并形成假菊形团或Homer-Wright菊形团,核分裂象多见.免疫组化标记:3例肿瘤CD99、突触素和波形蛋白均为阳性.1例肾肿瘤Ki67阳性率＜5%,另1例80%阳性.3例病理诊断均为PNET.例1肾肿瘤患者未行化疗,于术后14个月复发死亡;例2肾肿瘤及例3膀胱肿瘤患者术后予以化疗,分别于术后4、6个月死亡.结论泌尿系统原发性PNET是一种少见的高度恶性软组织肿瘤,诊断主要依据病理形态学特征及免疫组化标记.目前治疗方法主要是手术加放、化疗.

Abstract：

Objective To explore the clinico-pathological features, immunophenotype, treatment and prognosis of urologic primary primitive neuroectodermal tumor (PNET). Methods The clinical data of 3 patients with urologic PNET were analyzed retrospectively. All patients were male, aged 29, 32 and 75 years respectively. Two of the lesions were located in the kidney, and the third was located in the bladder. The sizes of renal tumors were 7.7 cm×6.2 cm and 12.6 cm×9.4 cm respectively. Imaging examinations revealed a well-defined mass with inhomogeneous echo inside. The size of bladder tumor was 10.0 cm×10.0 cm. CT scan demonstrated irregular thickening of the bladder wall, and the density of the wall was inhomogeneous. In the 2 cases of renal PNET radical surgery was performed, while an emergency palliative surgery to remove a blood clot and biopsy were performed in the bladder PNET case. Results In light microscope, the tumors were characterized by uniform small round or oval cells and nest-like or dense sheet structures surrounded by sparse fibrovascular stroma. Homer-Wright rosettes or pseudorosettes were observed, as well as mitoses. Immunohistochemical study revealed that all cases showed positive staining for CD99, synaptophysin and vimentin. One of the renal tumor cells showed positive for CD56, and the other renal tumor and urocystic tumor cells were focally positive for chromogranin A. Additionally, in 1 of the cases of renal tumor there was a high positive rate of 80% for Ki67 staining while the other case showed less than 5%. All 3 cases were eventually diagnosed as PNET. The first renal tumor case was not treated with radiotherapy and chemotherapy postoperatively, and the patient died of recurrence 14 months after surgery. Both the second renal tumor case and the bladder tumor case underwent chemotherapy postoperatively, and they died 4 and 6 months after surgery respectively. Conclusions The urologic primary PNET is a very rare, highly malignant soft tissue tumor, and the diagnosis must be based on pathologic findings and immunohistochemical phenotypes. The multimodal treatment for urologic primary PNET consists of surgery, chemotherapy and radiotherapy.     

Pulmonary Ewing Sarcoma/Primitive Neuroectodermal Tumor: A Case Report and a Review of the Literature

Primary thoracic sarcomas are very rare. The most common intrathoracic variants are synovial sarcoma, angiosarcoma, leiomyosarcoma, rhabdomyosarcoma, and sarcomatoid mesothelioma. Although thoracic Ewing sarcoma/primitive neuroectodermal tumor (PNET) usually develops on the chest wall, there have been reports of primary Ewing sarcoma/PNET of the lung.We present the case of a 22-year-old woman with Ewing sarcoma/PNET diagnosed following histologic, immunohistochemical, and in situ hybridization studies of a bronchial biopsy specimen. Radiography, ventilation-perfusion scintigraphy, and a bone marrow biopsy confirmed that the tumor was not metastatic. The patient was started on a chemotherapy regimen of vincristine, actinomycin, cyclophosphamide, doxorubicin, ifosfamide, and etoposide and responded well. She is now being seen regularly at our outpatient clinic.     

A resected case of renal cell carcinoma with metastasis to pancreas]

We report a case of renal cell carcinoma with metastasis to the pancreas, treated by radical nephrectomy and total pancreatectomy. A 56-year-old man visited our hospital because of macrohematuria and right low backache. An intravenous pyelography, ultrasonography and a CT scan of the abdomen revealed right renal tumor at the upper portion, about 11 cm in diameter, but no abnormal findings of the pancreas. Aortic and celiac angiograms demonstrated multifocal lesions, 1 or 2 cm in size, compatible with a metastatic tumor in the region of the pancreas. The patient underwent right radical nephrectomy and open biopsy of the pancreas. The right renal tumor was histologically revealed to be renal cell carcinoma without nodal or venous extension. Histological examination of the pancreas biopsy specimen confirmed it to be a renal cell carcinoma metastatic to the pancreas. Therefore, he underwent total pancreatectomy 1 month after the previous surgery. Three months after the second surgery, a CT scan of the brain revealed metastasis to the pituitary gland. He is still under therapy.     

Metastatic primitive neuroectodermal tumor of the kidney in adults.

OBJECTIVE: Primitive neuroectodermal tumors (PNET) of the kidney are rare and highly aggressive malignancies. The purpose of our study was to present information about the management of patients with metastatic disease. METHODS: The records of 2 patients (30-year-old female and 32-year-old male) with metastatic PNET of the kidney were reviewed and our data compared with the literature. RESULTS: Neither clinical evaluation nor radiological methods allowed to distinguish PNET from renal cell carcinoma. Immunohistochemistry revealed strong positivity for CD99 in tumor 1 and weak positivity for NSE and vimentin in both tumors. In tumor 2, EWS/FLI1 translocation was detected by RT-PCR. Patient 1 underwent nephrectomy, seven cycles of polychemotherapy, two cycles of high-dose chemotherapy, autologous bone marrow rescue, radiotherapy of suspicious skeletal foci and is without evidence of recurrent disease 28 months after therapy. Patient 2 underwent six cycles of polychemotherapy, nephrectomy, high-dose chemotherapy with cyclophosphamide and abdominal radiotherapy. Because of relapse high-dose chemotherapy with stem cell rescue was not performed. He underwent three further cycles of polychemotherapy and died one year after diagnosis due to cerebral metastasis. CONCLUSIONS: The diagnosis of renal PNET must be considered in young patients with renal neoplasm, particularly those with advanced disease at presentation. Achieving exact diagnosis has important clinical consequences because polychemotherapy and high-dose chemotherapy may lead to dramatic tumor reduction or even complete remission.     

Metastatic renal tumor from the lung with regional lymph node involvement: a case report

We report a case of metastatic renal tumor from lung cancer. A 53-year-old man presented with gross hematuria 2 years after treatment of the primary lesion. Investigations suggested a metastatic tumor in the right kidney from the lung which was accompanied with regional lymph node metastasis. Surgical treatment was not performed because of his poor condition and the lymph node involvement. Therefore, the combination chemotherapy of 5-fluorouracil, vincristine and doxorubicin, OK-432 was applied. Although this conservative management was effective, he died of progression 6 months later and an autopsy confirmed the diagnosis of metastatic renal tumor.     

Complete remission of advanced renal cell carcinoma by chemotherapy and surgical treatment: a case report

A rare case of metastatic renal cell carcinoma which represented complete remission by chemotherapy and surgical treatment is presented. A 59-year-old female was admitted to our hospital because of general fatigue, weight loss and appetite loss. The diagnosis of right renal tumor metastasized to both lungs and extending into the inferior vena cava was made by radiographic findings. Because of very poor general condition the first choice of treatment was chemotherapy with cisdichlorodiamine platinum, adriamycin, cyclophosphamide, 1-(2-tetrahydrofuryl)-5-fluorouracil) (UFT), and OK432. Five months after the beginning of chemotherapy both lung coin lesions disappeared completely, and radical nephrectomy including venacavotomy and tumor thrombectomy was performed. At present, 6 months after the radical nephrectomy, she is free from the disease and complete remission has been obtained by oral administration of 400 mg/day UFT and 5.0 KE OK432 intracutaneous injection every week.     

IIB osteosarcoma. Current management, local control, and survival statistics--S?o Paulo, Brazil

Ninety-two patients with IIB osteosarcoma of the extremities were treated with intraarterial (IA) cisplatinum (CDDP) followed by surgery [amputation (61.6%) or resection with endoprosthesis (38.4%)]. Postoperative chemotherapy alternating adriamycin and CDDP was used. The total three-year survival was 62.1%, and the disease-free survival was 41.1%. The pathologic evaluation of the degree of tumor necrosis in response to the IA CDDP showed that in 53.2%, the necrosis was over 90%. The multivariate analysis of prognostic factors has shown that the highest survival was among females with tumors smaller than 15 cm. Patients with lesions equal to or larger than 15 cm were three times as likely to die of the disease. A second, more aggressive study is now underway, in which high dose methotrexate (HDMTX) is preoperatively combined with adriamycin and CDDP. Following operation, ifosfamide is added to the cases with a smaller degree of tumor necrosis, while the other group of patients will continue with HDMTX, in addition to CDDP and adriamycin (these last two drugs are used in both arms). Until now, complete remission has been achieved in 82% and 86%, respectively, with a follow-up examination varying from four to 26 months (average, 14 months). This is of extreme importance, because the majority of the authors' patients have tumors at initial evaluation larger than 10 cm in diameter.     

Growth rates of primary and metastatic lesions of renal cell carcinoma

BACKGROUND: The natural history and growth rate of renal cell carcinoma (RCC) have not yet been determined. The growth rates of primary lesions in incidentally found RCC were compared with those of metastatic lesions. METHODS: Sixteen patients who did not receive immediate surgical treatment for renal solid masses that were later proven to be RCC were reviewed retrospectively. All primary lesions of the 16 patients were found incidentally. For comparison, metastatic lesions were evaluated in another 16 patients with RCC. Of these, 11 underwent surgical treatment for the primary lesions. RESULTS: The growth rates of primary and metastatic lesions of RCC varied. They ranged from 0.10 to 1.35 cm/year for primary lesions and from 0.08 to 7.87 cm/year for metastatic lesions. The growth rate of primary lesions of incidentally found RCC was lower than that of metastatic lesions (P = 0.0159). The initial tumor diameter and pathological grade did not affect the growth rate of the primary lesion of incidentally found RCC. However, a close correlation was found between the growth rate of metastatic lesions and the pathological grade of the primary lesion in patients with metastasis. CONCLUSIONS: The growth rate of incidentally found RCC varied. Some patients with the disease may be candidates for 'watchful waiting' when an immediate surgical treatment is not indicated, but they should be selected with great caution.     

Tumors of the testis In the forefront of oncology: a review and preview

Seven patients with metastatic and/or recurrent transitional cell carcinoma of the renal pelvis treated with systemic chemotherapy are evaluated. The median age of the patients was fifty-five; 6 were men; 6 of the tumors were histologic grade III and Stage C at the time of nephroureterectomy. Most of the patients presented with a large recurrent mass at the original site of surgery in addition to bone and/or pulmonary metastases. Four patients were treated with CISCA combination chemotherapy (cis-platinum, cyclophosphamide, and doxorubicin), and responded with 1 partial remission, 1 less than partial remission, and 2 symptomatic improvement. Two patients were treated with FAM combination chemotherapy (5 fluorouracil, doxorubicin, and mitomycin C) and demonstrated an objective response. One patient had a less than partial remission (30 per cent) and the second patient a partial remission. The seventh patient was treated with alternating courses of CISCA and FAM and thus far has received 8 courses and is in complete remission. The median survival time of the 7 patients is seven months (range four to fourteen months). Transitional cell carcinoma of the renal pelvis is responsive to chemotherapy; therefore, trials are indicated.     

Large mixed epithelial and stromal tumor of the kidney masquerading as metastatic renal cell carcinoma

We report the case of a 39-year-old woman with a large right renal mass 20 cm in size with heterogeneous solid and cystic components as well as concurrent liver lesions suspicious for metastatic renal cell carcinoma. Surgical extirpation of the renal mass and liver lesions was performed laparoscopically with the pathological analysis revealing a rare renal neoplasm--mixed epithelial and stromal tumor of the kidney--and adenomas of the liver.     

Brain metastasis of bladder carcinoma after total cystectomy: a case report

A 74-year-old woman underwent total cystectomy with ureterostomy on March 1993 on a diagnosis of advanced bladder cancer. The pathological diagnosis was transitional carcinoma grade 3, pT3a pN0 pV1, pL2. Two courses of adjuvant chemotherapy with CDDP and MTX were added. Three years and 3 months later, she began to suffer from amnesia and hemiparalysis on her left side. Brain MRI examination revealed a solid tumor, 4 x 4 x 3 cm in size, in the right frontal lobe of the cerebrum. No other metastatic lesions were found. She underwent surgical resection of the tumor and subsequent irradiation to right frontal lobe in the cerebrum. Pathological examination confirmed its origin of bladder cancer. She has been enjoying a disease-free life for 3 years with minimal neurological symptoms.     

Adult soft tissue Ewing sarcoma or primitive neuroectodermal tumors: predictors of survival?

BACKGROUND: Ewing sarcoma (ES) is the second most common primary osseous malignancy in childhood and adolescence. The improvement in survival is primarily associated with the combination of surgery and chemotherapy. HYPOTHESIS: Little is known about the outcome of adults with soft tissue ES or primitive neuroectodermal tumors (PNET). Certain prognostic factors from soft tissue sarcomas (tumor size, tumor location, margin status, and initial presentation) in adults (>16 years) with ES/PNET will help to identify factors associated with outcome. METHODS: Between July 1, 1982, and June 30, 2000, we identified 59 adult patients with primary soft tissue ES/PNET. Clinicopathologic factors were correlated with the end points studied: patient factors, tumor factors, pathologic factors, status of surgical margins, adjuvant chemotherapy, and radiation therapy. RESULTS: There were 41 male and 18 female patients, with a median age of 27 years (range, 16-72 years). Median tumor size was 8 cm, with all lesions being high grade. The most common site was the trunk (n = 22), with an even distribution of retroperitoneal, pelvis, buttock, and lower extremity (all n = 5). The median follow-up was 29 months (range, 6-222 months), with local recurrence identified in 13 patients (22%), with a median time to recurrence of 15 months (range, 5-200 months). Overall 5-year survival was 60%. Initial presentation was the only predictor of long-term survival, with primary tumor-only presentation having a 5-year survival of 60% (median not reached) compared with primary tumor plus metastatic disease having a 5-year survival of 33% (median, 17 months) (P =.02). CONCLUSION: Initial presentation of disease represents the only predictor of survival identified in this small group of adult patients with ES/PNET.     

Primitiver neuroektodermaler Tumor der Niere

Primitive neuroectodermal Tumor (PNET) is a rare malignant tumor of young adult. Patients often present with the classical trias of renal cancer (pain, haematuria, palpable tumor). Specific radiological signs are missing, and therefore a PNET is often diagnosed postoperatively. It is characterized by the expression of MIC2, neural markers (Vimentin, S-100, Synaptophysin) and EWS/FLI1-translocation. The tumor is often diagnosed in advanced stage, and prognosis is poor despite of multimodal treatment including radical nephrectomy, polychemotherapy (vincristine, adriamycin, cyclophosphamid, etoposide, ifosfamide) and radiation in case of metastases or incomplete resection of primary tumor. We represent two cases of metastatic renal PNET in a 28- and 39-year-old patient. Due to the multimodal treatment a partial and a complete remission was achieved, respectively.     

Adults with Ewing's sarcoma/primitive neuroectodermal tumor: adverse effect of older age and primary extraosseous disease on outcome.

OBJECTIVE: To assess outcome and prognostic factors for survival of adults with Ewing's sarcoma/primitive neuroectodermal tumor (PNET). BACKGROUND: Ewing's sarcoma/PNET is a disease of childhood rarely seen in adults. Accordingly, there is a relative paucity of published literature pertaining to outcome for adults with this disease. METHODS: Between 1979 and 1996, 37 patients with newly diagnosed Ewing's sarcoma/PNET were evaluated and treated at the Adult Sarcoma Program at Dana-Farber Cancer Institute and Brigham & Women's Hospital. Twenty-six patients had localized disease at presentation and 11 had metastatic disease. All but two patients received multiagent chemotherapy. Local treatment consisted of surgery (7 patients), surgery and radiation therapy (19), radiation therapy (6), or no local treatment (5). Median follow-up for living patients was 100 months (range 8 to 199). RESULTS: The 5-year survival rate for the group overall was 37%+/-9%. The 5-year local control rate was 85%+/-7%. Significant favorable predictors for survival on univariate analysis included localized disease at presentation, primary origin in bone, primary size <8 cm, and a favorable objective response to chemotherapy. Patients with localized disease had a 5-year survival rate of 49%+/-11% compared with 0% for those with metastatic disease at presentation. Multivariate analysis showed three significant independent predictors for death: metastatic disease at presentation, primary origin in extraosseous tissue versus bone, and age 26 years or older. CONCLUSION: Adult patients with Ewing's sarcoma/PNET at highest risk for death are those who are older than 26 years and have metastatic disease or an extraosseous primary tumor. The development of novel therapies should target these high-risk groups.     

原发性肾脏类癌2例报告并文献复习

目的：探讨原发性肾脏类癌的临床特征及诊治方法。方法：总结2例原发性肾脏类癌患者的临床资料结合文献复习讨论。例1,女,48岁。因右腰酸痛1个月入院。B超示右肾中上极中等回声占位,边界清楚,内有血流信号。CT示右肾中上极10．0cm×7．5cm实性占位,增强后病灶均匀轻度强化。例2,男,44岁。体检发现左肾占岱40天入院。B超示左肾门处中等回声占位,边界清楚,内有血流信号。CT示左肾中极肾门处3．7cm×3．5cm实性占位,增强后病灶均匀轻度强化;其内伴-钙化点。结果：2例均行根治性肾切除术。病理检查镜下肿瘤细胞形态为多边形,嗜酸性颗粒状细胞质,边界不清,呈柱状或缎带状排列混合有实性巢状排列;细胞核呈园形大小一致,核分裂像难见。免疫组化：突触素、波形蛋白均为阳性。病理诊断为肾脏类癌伴淋巴结转移。例1术后30个月死于肝骨转移;例2术后随访26个月,未见肿瘤复发转移。结论：原发性肾脏类癌临床罕见,易发生淋巴结转移,确诊依靠病理学检查,根治性肾切除应为治疗原发性肾类癌的首选方法。     

Renal cell carcinomas arising from the allograft and bilateral native kidneys

Abstract:   We present the case of a young lady who developed renal cell carcinomas (RCC) in the allograft and bilateral native kidneys metachronously within one year. She received a living donor kidney transplantation from her father. A solid tumor of 4 cm in diameter was uncovered first in the allograft kidney 103 months after transplantation, and was treated with graftectomy. Six months after graftectomy, a right renal tumor measuring 3.5 cm and left renal tumors emerged in the native kidneys. She underwent laparoscopic right and left radical nephrectomy in separate sessions. The pathological diagnosis in the allograft and right renal tumors was clear cell RCC with eosinophilic cytoplasm and that in the left kidney was clear cell carcinoma. Fluorescence in situ hybridization and human leukocyte antigen typing showed that each tumor was most probably primary disease. She was free of disease 18 months postoperatively. This is the first report on RCC arising both in the allograft and bilateral native kidneys.     

Complete remission of lung and hepatic metastases from renal cell carcinoma by interferon alpha-2b therapy: a case report]

A right renal tumor was found in a 74-year-old man with multiple metastases to the lungs and liver. Tumor thrombus extending into the inferior vena cava and a right spermatic varicocele were also noted at the first visit. Interferon alpha-2b and interferon gamma were administered for treatment. Partial remission of lung metastases, complete remission of hepatic metastases, and disappearance of the varicocele occurred after 4, 6 and 8 weeks, respectively. Then the primary right renal tumor was resected. Although only interferon alpha-2b was continued twice weekly by self-injection, complete remission of the lung metastases was obtained 13 weeks after the initiation of therapy. No evidence of recurrence or new metastasis has been found after 18 months. These results indicate that even advanced renal cell carcinoma may show a rapid response to interferon alpha. Interferon alpha is worth trying for metastatic renal cell carcinoma and should be continued for at least a few months.     

Renal cell carcinoma with a huge solitary metastasis to the contralateral adrenal gland: A case report

Renal cell carcinoma (RCC) is capable of metastasizing to several organs. Synchronous isolated contralateral adrenal metastasis of the primary RCC is, however, very rare. Herein we report a case of RCC with a huge solitary metastasis to the contralateral adrenal gland that was surgically treated. We scheduled nephrectomy for the left primary RCC and adrenalectomy for the right adrenal tumor. However, at surgery we found a huge right adrenal tumor that had invaded the right kidney, right renal vein, and inferior vena cava. Therefore right nephrectomy was performed simultaneously with resection and reconstruction of the inferior vena cava. Pathological findings demonstrated that the left renal tumor and right adrenal tumor had the same histology. Although the patient required hemodialysis, he remains well at six months postoperatively. So far, there have been only two cases of a solitary contralateral metastatic adrenal tumor that was larger than the primary RCC, thus the present case is the third one.     

Therapeutic experience of advanced urothelial tract tumors

Twenty-three patients with locally advanced and/or metastatic urothelial tumors were treated by surgery, chemotherapy or radiation. Radical operation with or without radiation resulted in tumor free conditions in 8 patients. Fifteen patients who had a few dimensionally estimable disease parameters, underwent chemotherapy. Objective responses were obtained in 6 of the 15 patients (1 CR and 5 PR). The objective response of low grade or low stage tumors or cases with high Karnofsky performance ratings was moderate. Combination chemotherapy with cisplatin (CDDP), doxorubicin (ADM) and cyclophosphamide or a CDDP-containing combination appeared to be beneficial for patients with advanced urothelial tract tumors with palliative purpose.