An aggressive course of Xp11 translocation renal cell carcinoma in a 28-year-old man

Abstract:   Cases of renal cell carcinoma (RCC) associated with Xp11 translocations are rare and are reported predominantly in children. We report a case of a young man who developed an aggressive Xp11 translocation RCC. A 28-year-old man presented with back pain, fever and macroscopic hematuria. Computed tomography of the abdomen showed a heterogeneous mass in the left kidney. Left radical nephrectomy was performed. Hematoxylin–eosin staining revealed nested and papillary architecture, clear and eosinophilic cytoplasm and vesicles with prominent nucleoli. Immunohistochemical evaluation revealed that the tumor cells showed nuclear labeling for TFE3 protein. On the basis of these findings, the case was diagnosed as Xp11 translocation RCC. This tumor massively recurred and led to the patient's death 2 years after the initial diagnosis. The utility of immunohistochemistry using antibodies against TFE3 in RCC occurring in young adults may be necessary for accurate diagnosis.     

Clinicopathological factors predicting recurrence of N0M0 renal cell carcinoma: A case series analysis of one facility

BACKGROUND: Although many factors have been reported as predictors of the recurrence of renal cell carcinoma (RCC), none of the factors are consistent among different studies. In the study presented here, the potential clinicopathological predictors of the recurrence of N0M0 RCC were examined. METHODS: A total of 201 patients who underwent nephrectomy for N0M0 RCC were examined to determine the pathological tumor stage (pT stage), pathological tumor grade of malignancy (tumor grade), symptoms, and tumor size. RESULTS: RCC recurred in 29 patients (14.4%), 50% of whom developed new tumors within 24 months after nephrectomy. The disease-free 3- and 10-year survival rates declined as the pT stage and tumor grade increased: these rates were, respectively, 98.6% and 86.5% for pT1a; 93.7% and 87.9% for pT1b; 100% and 100% for pT2; 78.6% and 58.0% for pT3a; and 88.9% and 16.7% for pT3b. Significant differences in the recurrence rate were noted between pT3 and pT1 or pT2, as well as between grade 3 disease and grade 1 or grade 2 tumors. Multivariate analysis showed that a combination of the pT stage, grade, and presence of symptoms was useful for predicting the recurrence of RCC. CONCLUSION: The present study showed that patients undergoing nephrectomy for N0M0 RCC should be followed-up carefully for 2 years postoperatively with special attention to high pT stage, high grade, and the development of symptoms.     

Induction of tumor regression by passive transfer of antibody from mice vaccinated with anti-idiotype antibodies resembling a human renal cell carcinoma-associated antigen

We have shown that six different internal image antiidiotype antibodies (Ab2) raised against the combining site of the murine monoclonal antibody G250 (MAbG250; Abl), which specifically reacts with a human renal cell carcinoma (RCC)-associated antigen, induce antigen specific humoral and cellular responses in mice. These six Ab2 can be divided into four mutually exclusive groups: (1) NUH31 and NUH51, (2) NUH44 and NUH82, (3) NUH71, and (4) NUH91. Immunization with NUH82 or NUH91 resulted in Ab3 sera that gave complete protection against tumor challenge. In this study, we tested the antitumor efficacy of NUH82- and NUH91-induced mouse sera (Ab3 sera; Ab3-82 and Ab3-91) in mice with established subcutaneous human RCC xenografts. Mice were treated 3 times per week by intraperitoneal injection of Ab3 sera (0.2 ml) or MAbG250 (250 μg) for 6 weeks. Treatment of NU12 human RCC xenografts of approximately 20 mm(3) expressed as tumor size index (TSI) with NUH-Ab3 sera or MAbG250 resulted in significant tumor growth inhibition compared with tumors treated with Ab3 sera from mice immunized with control immunoglobulin (Ab3-MOPC). In all Ab3-NUH treated mice, tumors stabilized or disappeared completely. In contrast, Ab3-MOPC treatment did not result in any antitumor effects. Tumor remnants in Ab3-NUH treated animals contained viable tumor cells surrounded by infiltrating mouse cells, whereas no infiltration was observed in control tumors. These findings demonstrate that Ab3 sera obtained from NUH82- or NUH91-immunized mice are very effective in eradicating established RCC [i.e., Ab2 vaccination may be able to eradicate (minimal) residual disease in RCC patients].     

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.     

Thymoma which is detected with a cough derived from pulmonary invasion; report of a case

A 83-year-old female was referred to our hospital for investigation of a persistent cough. A chest X-ray showed enlargement of the mediastinum. Computed tomography of the chest showed an anterior mediastinal mass with a maximal diameter of 6.5 cm, which had invasion to the lung. The patient underwent thymothymectomy combined resections of the lung, pericardium, and left innominate vein through a median sternotomy. Histological examination of the resected tumor revealed a World Health Organization (WHO) classification type B3 thymoma with infiltration into the lung. There were no infiltrations of the tumor into the pericardium and the innominate vein. A persistent cough disappeared after surgery. No adjuvant chemotherapy was performed. She is still free from disease with a follow-up period of 4 months. We report a rare case of thymoma detected with a persistent cough derived from pulmonary invasion.     

Enhancement of T cell proliferative response against autologous cancer cells of a metasatic renal cell carcinoma patient after unexplained regression

The unexplained regression of a metastasis of renal cell carcinoma (RCC) is rare phenomenon. While an immune mechanism has been proposed for spontaneous or unexplained regression of RCC, only limited data are available regarding immunological response. We report a case of a 62-year-old man with RCC whose paravertebral pleural tumor regressed after the withdrawal of interferon-alpha. In the present case, we demonstrate the enhancement of T cell proliferative response against autologous RCC cells secondary to the unexplained regression using the mixed-lymphocyte tumor culture. To our knowledge, the enhancement of an antitumor immunity secondary to an unexplained regression of RCC has not previously been reported.     

Renal cell carcinoma and paraneoplastic IgA nephropathy.

Paraneoplastic nephropathy is rarely associated with human tumors. Little is known about the pathogenetic background of this relationship. To our knowledge, no conclusive study of the association of potentially 'immunogenic' renal cell carcinoma (RCC) and paraneoplastic nephropathy has been published. For this reason, we performed an immunohistochemical analysis of native resected kidneys of 60 patients with RCC, paying special attention to their pre- and postoperative records. Sixteen (27%) of the 60 tumor patients had immune complex nephropathy (11 IgA nephropathy [IgA NP] and 5 focal segmental glomerulosclerosis [FSGS]). Preoperative proteinuria and/or hematuria observed in 11 of 16 cases disappeared in 6 IgA NP patients within a 2- to 3-month follow-up after nephrectomy. Eleven of 16 tumors stained with the anti human immunoglobulin (IgA or IgM) of the same isotype as that present in glomerular immune complexes. In 3 IgA NP patients RCC-associated von Hippel-Lindau (VHL) protein and IgA staining were found simultaneously in the tumor and glomeruli, with the clinical and laboratory findings disappearing after nephrectomy. Immune injury of the glomeruli due to a tumor-induced antigen-antibody response was demonstrated in these 3 IgA NP patients.     

Effect of papillary and chromophobe cell type on disease-free survival after nephrectomy for renal cell carcinoma

Background The clinical staging of renal cortical tumors traditionally has not evaluated the potential effect of histological subtypes on survival. Evidence suggests that conventional clear cell renal cell carcinoma (RCC) and nonconventional clear cell RCC (chromophobe and papillary) have different metastatic potential. Using a large renal tumor database, we examined the effect of tumor histology on the pattern of metastasis and patient survival. Methods All patients with nonmetastatic renal cortical tumors undergoing partial or radical nephrectomy were identified from a renal tumor database between July 1989 and July 2002. Kaplan-Meier and Cox regression tests were used for statistical analysis. Results Analysis revealed 1057 patients: 794 with conventional clear cell RCC, 157 with papillary RCC, and 106 with chromophobe RCC. Metastasis occurred in 95 conventional clear cell RCC, 9 papillary RCC, and 6 chromophobe RCC. Metastasis occurred in 95 conventional clear cell RCC, 9 papillary RCC, and 6 chromophobe RCC with a median follow-up of 34.6, 43.0, and 33.2 months, respectively. Using log-rank analysis, chromophobe and papillary RCC were associated with an improved disease-free survival at 5 years (P=.009 and .015, respectively). Multivariate analysis revealed tumor size, stage, and chromophobe histology as significant variables for disease progression. Conclusions Renal cortical tumors have distinct histological subtypes with varying degrees of metastatic potential. Conventional clear cell RCC, which comprises two thirds of renal cortical tumors presenting with localized disease, has a less favorable outcome when compared with papillary and chromophobe RCC. Controlling for size and stage, chromophobe, and not papillary, RCC was a significant variable for disease progression compared with conventional clear cell RCC. Knowledge of renal cortical tumor histological subtype is critical for projecting prognosis, tailoring follow-up strategies, and designing clinical trials. Presented at the 56th Annual Cancer Symposium, Society of Surgical Oncology, San Diego, CA, March 5–9, 2003.     

Two cases of renal cell carcinoma that underwent radical nephrectomy subsequent to complete tumor enucleation]

We have reported the favorable therapeutic results of non-ischemic complete enucleation using a microwave tissue coagulator as a method of nephron-sparing surgery for small renal cell carcinoma (RCC). We experienced two elective cases that underwent translumbar nephrectomy subsequent to the tumor enucleation. The first case showed another RCC in a cyst, concomitant with the enucleated RCC. The second case was a pT3a spindle cell carcinoma with high-grade malignancy. We decided to nephrectomize these enucleated kidney after obtaining well-informed consent. Here we report these controversial cases and discuss about the indication and outcomes of complete tumor enucleation for small RCC.     

A case of anaphylactic shock induced by pirarubicin hydrochloride

A 75-year-old man was admitted to our hospital for treatment of superficial bladder tumor. Transurethral resection (TUR) was performed and histopathological examination revealed a transitional cell carcinoma (G2). Despite one course of post-TUR bladder instillation therapy using pirarubicin hydrochloride, carcinoma in situ (CIS) was found 4 months later. CIS disappeared after another course of bladder instillation therapy using BCG; but, it recurred a month later. BCG bladder instillation therapy was performed again, and no malignant cells were detected in the urinary tract thereafter. Four months later, lung metastasis was diagnosed and an MVAC regimen (cisplatin, methotrexate, vinblastin adriamycin) was administered. However, anaphylactic shock was induced by intravenous injection of pirarubicin hydrochloride, so this therapy was stopped in the middle of the second course. Even though the lung metastasis disappeared once after the same MVAC treatment, it recurred the following year. At that time, 3 courses of a cisplatin-methotrexate-vinblastin regimen were administered, and a complete response was achieved.     

A case of renal cell carcinoma after successful treatment of Wilms tumor

This case report documents the eighth reported case of renal cell carcinoma (RCC) occurring after treatment of Wilms tumor. Although secondary malignancies after treatment of Wilms tumors are not infrequent, RCC as the second malignancy is rare. We discuss a 17-year-old girl whose RCC was diagnosed 12.5 years after diagnosis of a Wilms tumor. In addition, we review the literature on the subject. Renal cell carcinoma has been proposed as a consequent of chemoradiation; however, a genetic susceptibility must be considered. Because it is routine to assess the functional status of the remaining solitary kidney by annual ultrasonography, we recommend assessing for the presence of secondary renal malignancies and perhaps continuing routine ultrasounds long-term.     

CD70: a new tumor specific biomarker for renal cell carcinoma

PURPOSE: To date there have been no specific tumor markers available for the differential diagnosis of renal cell carcinoma (RCC). In an earlier study we identified high RNA expression of CD70 in clear cell RCC. CD70 is a type II transmembrane protein belonging to the tumor necrosis factor family. It represents the ligand for CD27, a glycosylated transmembrane protein of the tumor necrosis factor receptor family. To our knowledge the function of CD70 in solid tumors is not known. In the current study we analyzed CD70 protein expression in different RCC subtypes. MATERIALS AND METHODS: A total of 68 tumor samples of different histopathological subtypes were investigated by immunochemistry, including 41 clear cell, 19 papillary and 5 chromophobe RCCs, and 3 oncocytomas as well as their normal tissue counterparts. Immunochemistry was performed on frozen tissue samples using monoclonal antibody against CD70. RESULTS: None of the normal kidney tissues showed CD70 expression. In contrast, all clear cell RCCs expressed CD70 at a high level. Positive immunostaining was observed in 1 papillary (5%) and in 1 chromophobe (20%) RCC. Five papillary tumor samples (26%) showed focal staining in less than 5% of cells. All other samples were negative for CD70. CONCLUSIONS: Our study identified CD70 as a new specific tumor marker for clear cell RCC. This new marker can be used for differential diagnosis in cases of uncertain histological classification. The function of this protein in tumorigenesis and its use as a diagnostic marker in serum and urine or as a therapeutic tool must be investigated in further studies.     

Case Report: Octreotide as an Adjunct to Embolisation in the Management of Recurrent Bleeding Upper Gastrointestinal Metastases from Primary Renal Cell Cancer

The recommended treatment for patients with severe bleeding from upper gastrointestinal (GI) metastases is embolisation. We report a case in which despite adequate embolisation major haemorrhage from renal cell carcinoma (RCC) gastric metastases continually recurred. During a severe bleed refractory to embolisation octreotide was used to control and prevent further bleeding. No further episodes of severe haemorrhage occurred over the following 23 months since starting treatment. Octreotide has been observed to be effective both in the acute management of persistent haemorrhage and in prevention of subsequent haematemesis from GI metastatic RCC.     

Chromophobe renal cell carcinoma and 'capsulomas' with acquired cystic disease of the kidney in a long-term hemodialysis patient

Abstract:   Patients receiving long-term hemodialysis tend to develop renal cell carcinoma (RCC). Among such cases, chromophobe RCC and so-called 'capsulomas' are rarely reported. Here, we report a case of a Japanese woman in her early 70s, who developed both renal lesions after 17 years of hemodialysis. The patient received radical nephrectomy for enlarging renal mass. Grossly, the resected kidney showed a dominant tumor and small-sized subcapsular nodules. Histologically, two types of neoplasm, chromophobe RCC and 'capsuloma', existed with acquired cystic disease of the kidney. Chromophobe RCC had eosinophilic cytoplasm with perinuclear halos, and some tumor cells showed oncocytic features. Hale's colloidal iron staining showed a weakly positive cytoplasmic reaction. Immunohistochemistry was diffusely positive for cytokeratin 7, but negative for vimentin in the tumor cells. 'Capsulomas' were multiple subcapsular nodules composed almost entirely of smooth muscle-like cells with immunoreactivity for melanosome-associated antigen detected by HMB-45.     

Neurologic toxicity associated with interferon α therapy for renal cell carcinoma

A 67-year-old man received interferon alpha (IFN alpha) therapy for lung metastases of renal cell carcinoma (RCC). Multiple pulmonary metastases disappeared completely. However, neurological toxicity was detected by magnetic resonance imaging (MRI) as abnormal brain lesions. After discontinuation of IFN alpha therapy, his neurological symptoms and abnormal lesions on MRI disappeared completely. Complete remission of RCC has continued, and results of neurological study have remained normal for 5 years after discontinuation of IFN alpha therapy.     

Metastatic angiosarcoma of the brain

Two patients with metastatic angiosarcoma of the brain are described. In one, a 17-year-old man, the tumor was located at the pineal region and exhibited significant vascularity. It was sensitive to radiation therapy and disappeared after radiation of 50 Gy; however, it recurred after 1 year and a new lesion was found in the liver. The other patient is a 31-year-old woman who experienced sudden onset of headache. Computed tomography scan revealed three separate masses in the brain. One tumor was surgically removed. The other two were sensitive to radiation therapy and disappeared after radiation of 40 Gy. A new lesion was found in the femur 16 months after the operation. Brain metastasis from angiosarcoma is exceedingly rare. Both patients developed symptoms with intracranial hemorrhage. The diagnosis of the metastatic lesions preceded diagnosis of the primary lesion by 12 and 16 months, respectively. Ulex europaeus 1 lectin and factor VIII were very useful in establishing the diagnosis of angiosarcoma.     

Renal cell carcinoma with concomitant solid pseudopapillary tumor of the pancreas: A case report

INTRODUCTIONSolid pseudopapillary tumor (SPT) of pancreas is an unusual low-grade malignant epithelial tumor that usually occurs in young women and can be treated with surgical resection. Renal cell carcinoma (RCC) is the most common solid lesion of the kidney and primarily a disease of the elderly patient.PRESENTATION OF CASEIn this article we present a case of RCC with concomitant SPT of the pancreas who was treated successfully with a radical nephrectomy and distal pancreatectomy.DISCUSSIONRCC with concomitant SPT may associated in β-catenin gene mutation. But no prior reports have described RCC with concomitant SPT of the pancreas in the same patient.CONCLUSIONTo the best of our knowledge, this is the first report of RCC with concomitant SPT of the pancreas in the same patient.