Varicocele complicating spontaneous arteriovenous fistula.

We describe herein a rare case of a varicocele complicating spontaneous arteriovenous fistula. A 40-year-old man was referred to our hospital in November 2006, complaining of a non-tender mass in the left scrotum at the age of 15 and thereafter. On examination, his left scrotum revealed a large varicocele, but no manifest superficial thrill was noted. Scrotal ultrasonograpy revealed approximately 7 cm large varicocele. Computed tomography angiography revealed the existence of an arteriovenous fistula between the left testicular artery and the veins of the left pampiniform plexus. We laparoscopically carried out internal spermatic vessels ligation under the diagnosis of a varicocele complicating a spontaneous arteriovenous fistula. The postoperative course was uneventful. At 18 months postoperatively, the varicocele and fistula had not recurred.

Primary choriocarcinoma of the urinary bladder.

A 72-year-old woman with asymptomatic macrohematuria was referred to our hospital. Cystoscopy revealed a 7 cm sessile tumor on the left lateral wall of the bladder. Subsequently an intravenous pyelography revealed left hydronephrosis. We performed transurethral biopsy and resection of the bladder tumor under the diagnosis of ordinary malignant bladder tumor. Histopathologically, the lesion was shown to be an undiffentiated urothelial carcinoma, G3, > or = pT2, containing syncytiotrophoblastic giant cells. The level of serum human chorionic gonadotropin-beta (hCG-beta) level was slightly elevated (0.3 ng/ml; normal value: < 0.1). Because a further examination revealed an invasion into the surrounding fat tissue of the bladder and left ureter, a total cystohysterectomy with an ileal conduit were performed. The final histopathological classification was choriocarcinoma of the urinary bladder, pT3a, pN1, pMx. An adjuvant combination chemotherapy was carried out using methotrexate, vinblastine, adriamycin and cisplatin (MVAC). After two courses of chemotherapy, the serum hCG-beta levels returned to normal. Eleven months postoperatively, however, there was evidence of multiple lung metastases. The patient died 12 months after the surgery as a result of complications caused by widespread metastases.

A case of retroperitoneal bronchogenic cyst treated by laparoscopic surgery.

We describe herein a rare case of a retroperitoneal bronchogenic cyst successfully treated by laparoscopic surgery. A 39-year-old man with low-grade fever was referred to our hospital because of suspicion of an adrenal tumor. Abdominal computerized tomography (CT) and ultrasonography revealed a homogenous solid mass, 35 x 30 mm in diameter, in the left suprarenal region. Laboratory studies showed that the levels of adrenal hormones were normal except for the white blood cell count of 9,700/microL and C-reactive protein of 1.7 mg/dl. We diagnosed it as a non-functioning adrenocortical adenoma or an adrenal cyst. However, one year later he underwent laparoscopic surgery because the mass had gradually increased by 10 mm and the low-grade fever persisted. Pathological evaluation of the surgical specimens established the diagnosis of retroperitoneal bronchogenic cyst. The low-grade fever disappeared after the surgery.

[Case of local recurrence of the periurethral proximal-type epithelioid sarcoma successfully treated by repeated regional excision].

Proximal-type epithelioid sarcomas are rare soft tissue neoplasms occuring in the soma or thigh and often repeat recurrence and metastasis. We present a case of locally recurrenced proximal-type epithelioid sarcoma that could be treated by regional excision alone. A 62-year-old man visited our institute for a growing mass in the perineal region. Computed tomography (CT) showed a periurethral tumor 22 x 13 mm in diameter in the perineal region. The tumor was excised regionally, and the pathological examination with immunohistochemical staining revealed that the tumor was proximal-type epithelioid sarcoma. Local recurrence of the tumor occurred 2 years 7 months later without any metastatic lesion, and regional excision was performed again. Pathological diagnosis was proximal type epithelioid sarcoma and it was identical to the primary tumor. The patient is free of the disease 1 year after the second surgery of the tumor.

[A case of adrenocortical adenoma coexisting with gastrointestinal stromal tumor].

A 48-year-old man was referred to our institute for the evaluation of a concomitant gastric submucosal tumor and right adrenal tumor, incidentally found by ultrasound examination. Computed tomography showed a mass with a diameter of 6 cm adjacent to the stomach and the right adrenal tumor with a diameter of 3 cm. These tumors had similar characteristics in both plain and enhanced imagings. By magnetic resonance imaging, the intensity of the right adrenal tumor was equivalent to the liver in both T1 and T2 weighted images. On the other hand, the gastric submucosal tumor showed low intensity in T1 weighted images and high intensity in T2 weighted images. An adosterol scintigram showed slight accumulation at the region of adrenal tumor. The results of all conducted serum and urinary hormonal examinations were found to be within the normal range. Adrenalectomy and partial gastrectomy were performed laparoscopically. Pathological diagnosis of the adrenal tumor was a cortical adenoma, and that of the gastric submucosal tumor was gastrointestinal stromal tumor (GIST). The gastric tumor was immunohistochemically stained positive with the C-kit and CD34 and negative for s-100 protein and desmin. Histopathological diagnosis was coincident with gastric GIST and right adrenocortical adenoma, and the GIST was diagnosed as a high risk tumor because its diameter was over 5 cm.

Primary symptomatic perivesical paraganglioma: a case report.

Paraganglioma, extra-adrenal pheochromocytomas, are relatively rare in adults, with most arising from para-aortic sympathetic and visceral organs, such as the bladder. Paraganglioma localized at the extravesical retroperitoneal pelvic cavity is extremely rare. We report a case of symptomatic perivesical pheochromocytoma in a 34-year-old man treated by surgical excision. Symptoms related to cathecolamine secretion ceased after surgery, and the patient has remained disease-free for 24 months.

Retroperitoneoscopic adrenalectomy for adrenal tumors via a single large port.

BACKGROUND AND PURPOSE: Laparoscopic adrenalectomy is generally performed with carbon dioxide insufflation of the cavity and requires multiple trocars. This study reports the outcomes of retroperitoneoscopic adrenalectomy (RA) for adrenal tumors via a single port using a large cylinder without carbon dioxide insufflation. PATIENTS AND METHODS: Fifty-four patients with adrenal tumors were treated using RA via a single large port. The average tumor size was 2.6 cm. For surgery, patients were placed in the lateral decubitus position with slight flexion, and a 4.5-cm skin incision was performed below the 12th rib in the midaxillary line. The retroperitoneal space was dissected using index fingers and a balloon dilator. A rectoscope tube with a 4-cm diameter was inserted, and the adrenal glands were removed endoscopically via the single large port without carbon dioxide insufflation. RESULTS: This procedure was completed in 53 patients (98.1%). The average duration of surgery was 203 minutes, and the mean estimated blood loss was 252 mL. Four patients (7.4%) required blood transfusion. Postoperative major complications, including fulminant hepatitis and pulmonary thrombosis, were observed in two patients (3.7%), and the patient with hepatic disease died on the 14th postoperative day. The mortality rate after surgery thus was 1.9%. However, no local tumor recurrence or hormonal relapse has occurred at a median follow-up of 34 months. CONCLUSIONS: This procedure appears to be effective and relatively minimally invasive. However, it is limited by the narrow working space and restriction of the manipulation of instruments.

Expression and regulation of tumor suppressor gene maspin in human bladder cancer.

Maspin is a member of serine protease inhibitor family with tumor suppressing activity for breast and prostate cancers, acting at the level of tumor invasion and metastasis. However, there have been no published data regarding the role of maspin in human bladder cancer. We evaluated maspin expression in 65 series of bladder cancer samples (22 transurethral resection (TUR) and 43 radical cystectomy) and studied the regulatory mechanism of maspin gene activation in bladder cancer cells. Maspin expression was immunohistochemically detected in four (18.2%) patients with TUR and 22 (51.2%) patients with radical cystectomy whereas no expression was observed in normal transitional cells located at tumor-free area in bladder. The maspin expression was significantly correlated with the development of muscle invasive bladder cancer (P=0.00008). Using a luciferase reporter system, maspin promoter activity was induced in the maspin-positive bladder cancer cell lines as well as maspin-negative RT4 cells. Furthermore, treatment with the DNA methyltransferase inhibitor, 5-aza-2' deoxycytidine, and histone deacetylase inhibitor, trichostatin A, led to re-expression of maspin in RT4 cells. Our results indicate that maspin may contribute to bladder cancer development and that DNA methylation and histone deacetylation may be important for regulating maspin gene activation in bladder cancer cells.

[Adjuvant hormone therapy in patients with positive surgical margins after radical prostatectomy].

BACKGROUND: We carried out a retrospective study comparing radical prostatectomy plus adjuvant hormone therapy with radical prostatectomy plus surveillance in patients with positive surgical margins to evaluate whether adjuvant hormone therapy is beneficial for disease free survival. PATIENTS AND METHODS: Sixty-five patients with positive surgical margins after radical prostatectomy were included in this study. Twenty-six patients received adjuvant hormone therapy. Thirty-nine patients underwent surveillance with salvage hormone therapy at PSA failure. None of these 65 received androgen deprivation prior to surgery. Treatment outcomes were measured in terms of progression free survival. RESULTS: Five year clinical progression free survival rates for the patients with positive surgical margins in the adjuvant therapy group and surveillance group were 85.9% and 80.0% respectively (p = 0.85). Clinical progression free survival between the groups was not statistically different in terms of seminal vesicle involvement and tumor grade. The difference of clinical progression free survival between the two groups approached statistical significance in poorly differentiated tumor (p = 0.08). CONCLUSIONS: We conclude that adjuvant hormone therapy is not beneficial in terms of progression free survival in patients with positive surgical margins. Nevertheless, adjuvant hormone therapy could be beneficial in patients with poorly differentiated prostate cancer.

Case of seminoma with possible spontaneous regression without extragonadal metastatic lesion.

We present an extraordinarily rare finding of a testicular seminoma apparently in the process of spontaneous regression without accompanying metastatic lesion. A 35-year-old man visited our institute for persistent scrotal induration after the administration of antibiotics for several weeks. Ultrasonography and magnetic resonance imaging showed a capsulized tumor in his left atrophic testicle, without other visceral tumor or lymphadenopathy. We performed left radical orchiectomy. Histological examination revealed a fibrotic tumor with a small area of pure seminoma apparently undergoing spontaneous regression, and an intratubular germ cell neoplasia, unclassified, occurring diffusely. The patient is free of disease at 1 year after surgery.

Implications of circulating chromogranin A in prostate cancer.

OBJECTIVE: To evaluate whether measurement of circulating chromogranin A (CgA) levels provides clinicopathological and prognostic information in prostate cancer. MATERIAL AND METHODS: Plasma CgA levels were measured in 57 patients with histologically confirmed prostate cancer (stage B or less, n=22; stage C, n=10; stage D1, n=2; hormone-naive D2, n=12; hormone-refractory D2, n=11) and in 22 with undetected prostate cancer using an enzyme-linked immunoabsorbent assay. RESULTS: Median plasma CgA levels were significantly higher in patients with prostate cancer than in those with undetected cancer (p=0.0271). Higher stage (p<0.0001) and higher grade (p=0.0412) tumours were also significantly associated with higher plasma CgA levels. Above-normal CgA levels were also detected in 4/27 patients (15%) who underwent radical prostatectomy. Postoperative clinical failure was not reported in the prostatectomy patients; however, prostate-specific antigen (PSA) failure was reported in 44% of patients after a median follow-up period of 20.3 months. Multivariate analysis revealed that the pathological stage of the tumour was the only independent predictive variable for postoperative PSA failure (p=0.0494). Preoperative plasma CgA levels had no impact on postoperative PSA failure in the subgroup (prostatectomy patients). Elevated plasma CgA levels were associated with a poor survival prognosis in patients with stage D2 prostate cancer after a median follow-up period of 22.5 months (p=0.0416). CONCLUSIONS: It was demonstrated in this study that plasma CgA levels in prostate cancer increase with the severity of the disease, especially for progressive hormone-refractory prostate cancer (HRPC), after hormone therapy. Although this cross-sectional study involved only a small number of patients, we believe that plasma CgA levels may effectively predict HRPC status and prognosis in metastatic cases.

Fluorescence in situ hybridization analysis of c-myc amplification in stage TNM prostate cancer in Japanese patients.

OBJECTIVE: Genetic aberration such as the amplification of c-myc has been commonly found in advanced prostate cancer. The aim of this study was to elucidate chromosome 8 alteration, including a gain and amplification of 8q24 (c-myc gene), related to the progression and survival in advanced (Stage C) prostate cancer. MATERIALS AND METHODS: We used dual-probe fluorescence in situ hybridization with a centromere-specific probe for chromosome 8 (8cen), and with a region-specific probe for c-myc (8q24) to evaluate genetic changes in tumor samples from 50 patients who had undergone radical retropubic prostatectomy from 1986 to 2001. RESULTS: We classified the 8cen and c-myc copy numbers as normal, gain and amplification. The carcinoma foci with extra copies of c-myc, which was defined in 35 cases (70%), were divided into two groups: (a) a simple gain of the whole chromosome 8 (no increase in the c-myc copy number relative to the chromosome 8 centromere), which was identified in 15 cases (30%); and (b) a substantial amplification of c-myc (additional increases [AI] in the c-myc copy number relative to the chromosome 8 centromere), which was detected in 20 cases (40%). AI-c-myc was strongly associated with higher histopathological grades and Gleason's scores (P = 0.0330, 0.0190, respectively). Patients with the AI-c-myc had earlier disease progression (P = 0.0029) and earlier cancer death (P = 0.0087) than did patients with normal patterns. CONCLUSION: Identification of an AI-c-myc may serve as a potential marker of prostate cancer progression.

Immunohistochemical and ultrastructural features of neuroendocrine differentiated carcinomas of the prostate: an immunoelectron microscopic study.

The purpose of this study was to further define the immunohistochemical and ultrastructural characteristics of neuroendocrine (NE) differentiated prostatic carcinomas. Seventy-seven specimens were obtained from prostatic carcinoma tumors during prostatectomy, transurethral resection of prostate or biopsy in 77 prostate cancer patients, and analyzed by immunohistochemical staining for chromogranin A (CgA). Nine of these tumors were also studied by elctron microscopy and 4 were examined by pre-embedding immunoelectron microscopy. CgA-stained cells were detected in 36 tumors (47%). Clinically advanced tumors or tumors with higher histological grades were associated with increased NE differentiation. Three of the tumors studied by electron microscopy contained cells showing unequivocal NE differentiation revealed by the presence of neurosecretory granules, while the poorly NE-differentiated malignant cells contained pleomorphic granules, which were lysosomal-like rather than NE-type granules. Immunoelectron microscopy demonstrated the presence of CgA immunoreactivity on the pleomorphic granules in the poorly differentiated malignant glands. This study suggests that NE-differentiated malignant cells in prostate cancer tissues may induce aggressive behavior in adjacent proliferating neoplastic cells via a paracrine mechanism.

Prospective study of estramustine phosphate for hormone refractory prostate cancer patients following androgen deprivation therapy.

INTRODUCTION: Estramustine phosphate (EMP) in combination with other cytotoxic agents has been widely used in clinical trials as an anti-tumor agent for the treatment of hormone-refractory prostate cancer (HRPC). However, few prospective studies have considered the efficacy of EMP monotherapy for HRPC patients following androgen-deprivation therapy (ADT), given the availability of methods to measure prostate-specific antigen (PSA) levels in the serum. We therefore initiated a prospective study to determine whether EMP is efficient for HRPC following ADT using changes in PSA levels as the major endpoint. METHODS: After a diagnosis of anti-androgen withdrawal syndrome had been excluded, 34 patients with HRPC who showed an elevated serum PSA level in 3 or more sequential tests following ADT were treated orally with 560 mg/day of EMP. The clinical stage and the median PSA value for inclusion in the study were D2 and 25.9 (range 6.5-540.8) ng/ml, respectively. Treatment was continued until evidence of disease progression reappeared or until severe adverse effects appeared. RESULTS: Of the 34 patients enrolled, 29 were evaluated, while the other 5 (15%) patients were discontinued due to severe gastrointestinal side effects. Seven of the 29 patients (24%) showed a decrease of 50% or greater in serum PSA levels from the initially elevated values, with the median duration of PSA response being 8.0 (range 2.2-18.8) months. Baseline PSA, hemoglobin, alkaline phosphatase, lactate dehydrogenase, performance status, and length of time of initial hormonal treatment did not correlate with the PSA response. With a median follow-up time of 20.0 (range 3.2-45.6) months, the cancer-specific survival rate at 2 years was 83% in the PSA responders and 44% in the non-responders. The PSA response was correlated with cancer-specific survival (p = 0.029). CONCLUSIONS: Following ADT one quarter of HRPC patients responded to EMP, with more than 50% of patients showing a decrease in PSA levels and an enhanced survival rate.

Neuroendocrine differentiation in hormone refractory prostate cancer following androgen deprivation therapy.

OBJECTIVE: To evaluate the relationship between neuroendocrine differentiation (NED) status and hormone refractory prostate cancer (HRPC) following hormone therapy based on immunohistochemical study. METHODS: Seventy-two prostate cancer specimens obtained at radical prostatectomy and 21 prostate cancer autopsy specimens from patients who died from HRPC after androgen deprivation therapy were examined for NED status using an antibody against chromogranin A. These specimens were classified into 3 arms: 38 radical prostatectomy specimens from patients with no neoadjuvant hormone therapy (Group 1); 34 from patients with neoadjuvant hormone therapy for 3 to 6 months (Group 2); and 21 autopsy specimens from patients with HRPC following androgen deprivation therapy for more than 1 year (Group 3). Staining of prostatic carcinoma was scored as: 0 = no staining; 1 = staining cells <10%; 2 = staining cells 10-20%; and 3 = staining cells >20%. Differences in scores among the groups were compared using the Kruskal-Wallis rank test. Multivariate analysis using a logistic regression model was performed to examine whether NED status was associated with pathological stage (pT), grade and group. RESULTS: Forty-nine (53%) tumors had CgA stained cells. NED status increased with longer duration of hormone therapy (p<0.0001). The mean staining score (and standard deviation) was 0.4+/-0.7 in Group 1, 0.7+/-0.7 in Group 2, and 1.4+/-1.1 in Group 3, respectively. By multivariate analysis Group 3 had a relative risk of 5.46 (95%CI 1.28-23.29) for NED compared to the other groups. But other variables were not related to NED. HRPC following Long-term hormonal therapy was the only independent predictor of NED. CONCLUSIONS: The results of this study demonstrated that NED status was significantly increased in patients with HRPC following long-term androgen deprivation therapy, but it could not be discriminate whether the increase of NED is attributable to condition of hormone refractoriness or long-term hormonal therapy.

Multicentric liposarcoma.

Abstract A 72-year-old man complaining of upper abdominal discomfort was diagnosed as having retroperitoneal liposarcoma by means of diagnostic imaging. He then underwent an operation. One mass existed on the curvatura ventriculi major, extending to the hilum splenicum and pressing back the pancreal head and body. There was another mass to the left of the first, situated on the ventral side of the left kidney. Also, another mass was intramurally found adjacent to the curvatura ventriculi major. Histologically, the mass on the curvatura ventriculi major ranged from the peritoneal cavity to the retroperitoneum Its intraperitoneal portion was classified as a differentiated lipoma-like type and the retroperitoneal mass was of mucous type. The mass on the left kidney was of a differentiated fibrosing type. The intramural mass in the gastric curvature was found to be a differentiated lipoma-like type. The patient has been under observation for 12 months and has shown no recurrence.