[A Case of Aldosterone-Producing Adrenocortical Carcinoma].

A 59-year-old man presented with high blood pressure, hypokalemia and muscle weakness. His aldosterone/renin ratio was high and plasma renin activity was low. Computed tomography (CT) showed a heterogeneous left adrenal mass. Primary aldosteronism was diagnosed and laparoscopic left adrenalectomy was performed. The pathological diagnosis was adrenocortical carcinoma with positive surgical margins. He underwent radiotherapy and mitotane as adjuvant therapies. Subsequently, CT revealed multiple metastases, in the liver and retroperitoneum. After six courses of EDP (a combination of etoposide, doxorubicin and cisplatin), CT showed widespread metastases in the retroperitoneum and he chose to receive the best supportive care. Aldosterone-producing adrenocortical carcinoma is exceedingly rare. To the best of our knowledge, only67 cases have been reported. Complete resection is needed to improve prognosis and this was not achieved in our case. We therefore recommend careful selection of the operative procedure.

[A Case of Bladder Plasmacytoma Diagnosed during Treatment of Multiple Myeloma].

A 78-year-old man was treated with Bortezomib, Lenalidomide, and Dexamethasone, for multiple myeloma. Two years after the start of treatment, the patient came to our department with a complaint of gross hematuria. Cystoscopy revealed a tumor on the left wall of the bladder. Urine cytology was negative. Magnetic resonance imaging (MRI) of the lower abdomen showed a slightly high signal on the T2-weighted image, indicating an intravesical mass lesion invading outside the bladder. Contrast-enhanced computed tomography (CT) also showed an intravesical mass and enlarged left external iliac lymph node swelling. Transurethral resection of bladder tumor was performed. The resection specimen showed tumor cells. The pathological examination revealed CD138 (+) and light-chain restriction. The patient was diagnosed with plasmacytoma. The patient was treated with radiation therapy for plasmacytoma of the bladder and surrounding lymph nodes, and then with daratumumab and dexamethasone for multiple myeloma for one year; however, the patient died because of worsening of multiple myeloma.

[A Case of Multiple Penile Schwannoma].

A 48-year-old man presented with a painless nodule on the right ventral aspect of the penile shaft root. The patient visited the hospital and complained that the mass was growing. On physical examinations, a 5 cm mass without tenderness was palpated. An isoechoic mass about 4 cm in size was observed on ultrasonography. Inhomogeneous high signal intensity was observed on T2-weighted magnetic resonance imaging. A needle biopsy revealed schwannoma. During surgery for excision of the tumor, another small tumor was detected both tumors were resected. Both tumors were diagnosed as schwannoma by pathological examination. Out of 40 cases of penile schwannoma reported, eight were of multiple tumors. In our case, it was difficult to detect the other small tumor by preoperative diagnosis. Therefore, we recommend careful observation during surgery.

[A Case of Ureteral Metastasis from Prostate Cancer].

Ureteral metastasis from prostate cancer is rare. The present case report describes an 83-year-old patient with distant metastasis of prostate cancer to the right ureter that caused hydronephrosis. Upon initial examination at our hospital, he presented with a high prostate-specific antigen (PSA) level of 10.0 ng/ml. He was diagnosed with prostate adenocarcinoma, with Gleason score of 10 (5＋5) and clinical staging of cT2aN0M0. Intensity-modulated radiation therapy (IMRT) was performed after 1 year and 7 months of androgen depriation therapy. At 1 year and 4 months after IMRT, PSA increased to 3.068 ng/ml. Computed tomography scan revealed right hydronephrosis and thickening of the right ureter. We could not identify obvious malignant cells on ureteroscopic biopsy, and right nephroureterectomy was performed. Pathological examination revealed ureteral metastasis of prostate cancer. Six months after nephroureterectomy, PSA increased to 3.037 ng/ml. He was diagnosed with castration-resistant prostate cancer and has been treated with enzalutamide.

[A Case of Primary Renal Angiosarcoma with a Rapidly Worsening Course].

A 69-year-old man presented with left flank pain and underwent a computed tomographic scan, which revealed a large left renal mass of 16×9×5 cm. A left nephrectomy was performed. Pathological analysis revealed that the tumor was consistent with angiosarcoma of the left kidney with a negative surgical margin. He received combination therapy with radiation therapy and four courses of chemotherapy with doxorubicin and ifosfamide. However, he died 10 months after surgery because of pleural dissemination and peritoneal dissemination. Primary renal angiosarcoma is an extremely rare and highly aggressive tumor with a dismal survival rate, and a satisfactory surgical margin is necessary for its successful treatment. If primary renal angiosarcoma is suspected, biopsy may be considered before surgery. Primary renal angiosarcoma treatment with combination therapy of surgery, radiotherapy, and chemotherapy by a specialist multidisciplinary team with experience and expertise in sarcoma is preferable. Development of therapy for angiosarcoma is awaited.

A case of bilateral emphysematous pyelonephritis caused by Candida albicans.

Emphysematous pyelonephritis (EPN) is a life-threatening renal infection characterized by the formation of gas within the renal parenchyma and collecting duct system, as well as perinephric tissues. We herein report a case of bilateral EPN accompanied by the urinary tract infection caused by spherical growth of Candida albicans in a patient with underlying diabetes mellitus and prostate cancer. The diagnosis was assisted by computed tomography, urography, and gram staining. Despite immediate percutaneous catheter drainage and a 4-week course of antifungal treatment, the C. albicans infection was refractory, as indicated by continuous isolation from the urine, and the patient eventually died. A local autopsy of urinary organs revealed C. albicans in the renal tissue. EPN caused by Candida species frequently occurs in men, and almost all cases have underlying poorly controlled diabetes. This condition is subject to delayed diagnosis, which may lead to extended disease and high mortality. Candida species should be considered as causative microorganism for refractory EPN in patients with poorly controlled diabetes who are receiving antibiotic treatment. Gram staining may contribute to an early diagnosis of EPN caused by Candida species, which may require long-term antifungal therapy.

[A Case of Isolated Peritoneal Metastasis from Prostate Cancer].

Peritoneal metastasis of prostate cancer is extremely rare, with only a few cases reported. A 78-yearold male patient was introduced to our hospital presenting with a prostate-specific antigen (PSA) level of 94.0 ng/ml at examination. He was diagnosed with poorly differentiated adenocarcinoma of the prostate, with a Gleasonscore of 9 (5＋4) at cT3bN0M0. Intensity-modulated radiation therapy was performed after 6 months of combined-androgen blockade (CAB) therapy. Twenty-one months later, several lymph node metastases were observed. With the resumptionof CAB therapy, PSA levels dropped and the multiple lymph node metastasis disappeared ; however, peritoneal metastasis was observed after 43 months. We performed a laparoscopic biopsy and our diagnosis after pathological evaluation was metastasis of the prostate cancer. He was treated with Enzalutamide.

[Two Cases of Inflammatory Myofibroblastic Tumor of the Urinary Bladder].

We describe two cases of inflammatory myofibroblastic tumor (IMT) of the urinary bladder. Case 1 : A 55-year-old man complained of urinary frequency. Transurethral biopsy of the bladder tumor was performed ; pathological examination revealed IMT. After steroid therapy for 1 year, the tumor was markedly reduced in size. Laparoscopic partial cystectomy was performed. The patient has now been free from recurrence for 3 years after the surgery. Case 2 : A 61-year-old man presented with gross hematuria. Transurethral biopsy of the bladder tumor revealed IMT. Despite steroid therapy for 6 months, the tumor size increased. Laparoscopic partial cystectomy and vesicoureteroneostomy were performed. The patient has now been free from recurrence for 1 year and 11 months after the surgery. IMT of the urinary bladder often responds well to steroid therapy. We experienced two cases of IMT. One showed a good response to steroid therapy and the other showed a poor response to steroid therapy. In both cases, we performed laparoscopic partial cyctectomy and the patients have remained free from recurrence IMT of the urinary bladder may have a good prognosis after complete surgical resection.

[Lymph Node Metastasis to the Prostatic Anterior Fat Pad in the Patients Treated with Prostatectomy].

We investigated the prostatic anterior fat pad (PAFP) for the presence of lymph nodes and lymph node metastases after excision during radical prostatectomy for the treatment of prostate cancer. From November 2013 to May 2017, 215 patients underwent radical prostatectomy for localized prostate cancer at our institution. PAFP was removed and sent for pathological analysis routinely to evaluate the presence of lymph nodes and lymph node metastases. Of the 215 patients, 20 (9.3%) had lymph nodes in the PAFP. Among the 20 patients with lymph nodes in the PAFP, 2 patients (10.0% ; 0.9% of all patients) were positive for metastatic prostate cancer. These patients did not have positive nodes elsewhere among the pelvic lymph nodes. There was no significant difference in the clinicopathological features between the patients with lymph nodes in the PAFP and those with no lymph nodes in the PAFP. The present findings are similar to those in the Western population. Considering the very low rate of lymph node metastasis in PAFP, it is necessary to discuss which patients should be selected for pathological analysis of PAFP.

Supratentorial extraventricular WHO grade III (anaplastic) ependymoma 17 years after total removal of WHO grade II ependymoma of the fourth ventricle.

We report a WHO grade III ependymoma of the supratentorial interhemispheric fissure and grew to form a large mass with anaplastic transformation without local recurrence 17 years after the total removal of a fourth ventricular WHO grade II ependymoma. We emphasize the necessity of long-term follow-up, even in benign ependymomas.

[A Case of Primary Sigmoid Colon Lymphoma Presenting with Sigmoidovesical Fistula in a Patient with a Long History of Ulcerative Colitis].

A 73-year-old male patient with a 16-year history of ulcerative colitis presented to our hospital with a history of pneumaturia and fever. Cystoscopy and a computed tomography scan showed sigmoidovesical fistula. Colonoscopy showed a necrotic tumor along with sigmoidovesical fistula. A biopsy was not sufficient to make a definitive diagnosis of the tumor. Total colectomy with ileostomy and partial cystectomy were performed. A pathological examination showed diffuse large B-cell lymphoma of the sigmoid colon. On postoperative day 35, delayed dehiscence of the bladder wall was detected and a biopsy of the bladder wall showed lymphoma. Standard systemic chemotherapy (R-THP-COP) was administered and the defect of the bladder was closed. Three years and 2 months postoperatively, the patient has no local recurrence or distant metastasis.

[A Case of ACD-Associated RCC with Lymph Node Metastasis and Contralateral Renal Carcinoma after Nephrectomy].

A 59-year-old man who had undergone hemodialysis for 13 years was diagnosed with left renal cell carcinoma (RCC),cT1aN0M0,in 2010. He had a laparoscopic left nephrectomy,and the pathological diagnosis at that time was clear cell carcinoma,pT1a (multifocal). At 1 year after surgery,he was diagnosed with a left renal hilar lymph node metastasis and a new right RCC,cT1aN0M0. Consequently,he underwent a right nephrectomy and retroperitoneal lymph node dissection in 2012. Pathologic diagnosis by the current classification of the right renal tumor was acquired cystic disease-associated renal cell carcinoma (ACD-associated RCC),and that of the left hilar lymph node was metastatic RCC with sarcomatoid change. According to the revised classification the pathological diagnosis of the left renal tumor was ACD-associated RCC. There has been no evidence of recurrence or metastasis for 3 years after the second operation. The specific classification of dialysis-related renal tumors and their characteristics should be standard knowledge for urologists.

Clinicopathological analysis of 17 primary cutaneous T-cell lymphoma of the γδ phenotype from Japan.

Primary cutaneous γδ T-cell lymphoma (PCGD-TCL) is an aggressive lymphoma consisting of clonal proliferation of mature activated γδ T-cells of a cytotoxic phenotype. Because primary cutaneous γδ T-cell lymphoma is a rare disease, there are few clinicopathological studies. In addition, T-cell receptor (TCR) γδ cells are typically immunostained in frozen sections or determined by TCRß negativity. We retrospectively analyzed 17 primary cutaneous T-cell lymphomas of the γδ phenotype (CTCL-γδ) in a clinicopathological and molecular study using paraffin-embedded sections. Among 17 patients, 11 had CTCL-γδ without subcutaneous panniculitis-like T-cell lymphoma (SPTCL) features and six had CTCL-γδ with SPTCL features. Immunophenotypically, some significant differences were found in CD8 and CD56 positivity between our patient series of CTCL-γδ patients with SPTCL features and SPTCL-γδ patients described in the previous literature. A univariate analysis of 17 CTCL-γδ patients showed that being more than 60 years old, presence of visceral organ involvement, and small-to-medium cell size were poor prognostic factors. In addition, the 5-year overall survival rate was 42.4% for the CTCL-γδ patients without SPTCL features and 80.0% for those with SPTCL features. Consequently, there was a strikingly significant difference in overall survival among SPTCL, CTCL-γδ with SPTCL features and CTCL-γδ without SPTCL features (P = 0.0005). Our data suggests that an indolent subgroup may exist in CTCL-γδ. Studies on more cases, including those from other countries, are warranted to delineate the clinicopathological features and the significance in these rare lymphomas.

Keratocystoma: A Distinctive Salivary Gland Neoplasm Characterized by RUNX2 Rearrangements.

Keratocystoma is a rare salivary gland lesion that has been reported primarily in children and young adults. Because of a scarcity of reported cases, very little is known about it, including its molecular underpinnings, biological potential, and histologic spectrum. Purported to be a benign neoplasm, keratocystoma bears a striking histologic resemblance to benign lesions like metaplastic Warthin tumor on one end of the spectrum and squamous cell carcinoma on the other end. This overlap can cause diagnostic confusion, and it raises questions about the boundaries and definition of keratocystoma as an entity. This study seeks to utilize molecular tools to evaluate the pathogenesis of keratocystoma as well as its relationship with its histologic mimics. On the basis of targeted RNA sequencing (RNA-seq) results on a sentinel case, RUNX2 break-apart fluorescence in situ hybridization (FISH) was successfully performed on 4 cases diagnosed as keratocystoma, as well as 13 cases originally diagnosed as tumors that morphologically resemble keratocystoma: 6 primary squamous cell carcinomas, 3 metaplastic/dysplastic Warthin tumors, 2 atypical squamous cysts, 1 proliferating trichilemmal tumor, and 1 cystadenoma. RNA-seq and/or reverse transcriptase-PCR were attempted on all FISH-positive cases. Seven cases were positive for RUNX2 rearrangement, including 3 of 4 tumors originally called keratocystoma, 2 of 2 called atypical squamous cyst, 1 of 1 called proliferating trichilemmal tumor, and 1 of 6 called squamous cell carcinoma. RNA-seq and/or reverse transcriptase-PCR identified IRF2BP2::RUNX2 in 6 of 7 cases; for the remaining case, the partner remains unknown. The cases positive for RUNX2 rearrangement arose in the parotid glands of 4 females and 3 males, ranging from 8 to 63 years old (mean, 25.4 years; median, 15 years). The RUNX2 -rearranged cases had a consistent histologic appearance: variably sized cysts lined by keratinizing squamous epithelium, plus scattered irregular squamous nests, with essentially no cellular atypia or mitotic activity. The background was fibrotic, often with patchy chronic inflammation and/or giant cell reaction. One case originally called squamous cell carcinoma was virtually identical to the other cases, except for a single focus of small nerve invasion. The FISH-negative case that was originally called keratocystoma had focal cuboidal and mucinous epithelium, which was not found in any FISH-positive cases. The tumors with RUNX2 rearrangement were all treated with surgery only, and for the 5 patients with follow-up, there were no recurrences or metastases (1 to 120 months), even for the case with perineural invasion. Our findings solidify that keratocystoma is a cystic neoplastic entity, one which appears to consistently harbor RUNX2 rearrangements, particularly IRF2BP2::RUNX2 . Having a diagnostic genetic marker now allows for a complete understanding of this rare tumor. They arise in the parotid gland and affect a wide age range. Keratocystoma has a consistent morphologic appearance, which includes large squamous-lined cysts that mimic benign processes like metaplastic Warthin tumor and also small, irregular nests that mimic squamous cell carcinoma. Indeed, RUNX2 analysis has considerable promise for resolving these differential diagnoses. Given that one RUNX2 -rearranged tumor had focal perineural invasion, it is unclear whether that finding is within the spectrum of keratocystoma or whether it could represent malignant transformation. Most important, all RUNX2 -rearranged cases behaved in a benign manner.

Myopericytoma in the corpus cavernosum of the penis: A case report of a rare disease.

Introduction: Myopericytomas usually occur in the extremities of older individuals; however, they also rarely occur in the penis. We report a case of myopericytoma in the corpus cavernosum of the penis and review the relevant literature. Case presentation: A 76-year-old man presented with a slow-growing painless nodule on the left side of the penis. On physical examination, a non-tender, 7-mm mass was palpable. This tumor showed inhomogeneous low signal intensity on T2-weighted magnetic resonance imaging. The mass was excised and a myopericytoma diagnosed by pathological examination of the operative specimen. Conclusion: We here report a rare case of myopericytoma in the corpus cavernosum of the penis. To the best of our knowledge, this is the second reported case of a myopericytoma in the penis and the first in the corpus cavernosum of the penis. Clinicians should keep this rare possibility in mind when investigating a mass in the penis.

Intraductal papillary-mucinous neoplasm of the pancreas penetrating to the stomach and the common bile duct.

CONTEXT: Intraductal papillary mucinous neoplasm (IPMN) of the pancreas occasionally penetrates to others organs. We present a case of IPMN penetrating to the stomach and the common bile duct. CASE REPORT: A 75-year-old man was admitted to the hospital because of epigastric pain. Computed tomography (CT) showed a papillary tumor protruding into the markedly dilated main pancreatic duct and splenic vein obstruction. The tumor was diagnosed as IPMN arising in the main duct, but he rejected surgery and he was followed without treatment. One year later, gastroduodenoscopy revealed gastropancreatic fistula and we were able to pass an endoscope through the fistula and directly examine the lumen of the main pancreatic duct and the papillary tumor adjacent to the fistula. Absence of malignant cells on histopathology suggested mechanical penetration rather than invasive penetration. CT showed splenic vein reperfusion due to decreased inner pressure of the main pancreatic duct. Two and a half years later, CT revealed biliopancreatic fistula formation. Endoscope biliary drainage was performed but failed. Despite jaundice, he is still ambulatory and seen in the clinic three years after the first admission. CONCLUSIONS: We have experienced a case of IPMN penetrating to the stomach and the common bile duct that has taken a slow course. It represents the importance of distinguishing mechanical penetration from invasive penetration as well as mechanical splenic vein obstruction from splenic vein invasion.

[A case report of primary small cell esophageal carcinoma responding successfully to radiochemotherapy].

Primary small cell esophageal carcinoma is a rare cancer with a poor prognosis, for which to date there is no recommended standard treatment. We present a 60-year-old male with this disease who was successfully managed by the combination of radiation and chemotherapy. The patient was referred to our hospital for liver dysfunction of unknown cause. On admission, he was diagnosed as having a primary small cell esophageal carcinoma and multifocal metastasis in the liver. He received cisplatin and 5-fluorouracil(CDDP 40mg/m2-days 1, 8, 5-FU 400 mg/m2-days 1-5, 8-12)and local radiation of 50 Gy for the esophagus, followed by 4 courses of chemotherapy the same as listed above. At the end of therapy, radiological findings showed that both primary and metastatic lesions completely disappeared. Unfortunately, he died of liver failure due to the recurrence of metastatic small cell carcinoma in the liver 13 months after the end of initial therapy. However, the primary cancer lesion had not recurred by that time. These findings suggest the beneficial effect of the combination of radiotherapy and chemotherapy for advanced-stage small cell esophageal carcinoma.

Hypersensitivity pneumonitis associated with inhalation of catechin-rich green tea extracts.

A 51-year-old man presented with fever and fatigue after 3.5 months of antituberculosis therapy. High-resolution computed tomography of his chest revealed new ground-glass opacities and poorly defined centrilobular nodules. He had undergone catechin inhalation for 1 month. We diagnosed hypersensitivity pneumonitis (HP) based on the clinical course, bronchoscopy and a challenge test. Cases of HP due to inhalation of extracted catechin powder are rare. Although it has many known positive attributes, it is necessary to be aware that catechin can cause HP.

[Complete response of lung metastasis to interferon-alpha therapy after radical nephrectomy for sarcomatoid renal cell carcinoma with Stauffer syndrome: a case report].

A 75-year-old man was admitted to our hospital with liver dysfunction and elevated C-reactive protein level. He was diagnosed with renal tumor by ultrasonography. Computed tomography revealed a renal cell carcinoma (cT3aN0M0) with Stauffer syndrome. Laparoscopic radical nephrectomy was performed. Histological findings indicated clear cell carcinoma with a sarcomatoid component. After 1 month, lung metastasis was detected on an X-ray film. Interferon-α was administered, and complete response was achieved 2 months later. He has shown no evidence of recurrence in 27 months.

Rapid recurrence of squamous cell carcinoma at a lymphocele after nephroureterectomy: A rare case report.

INTRODUCTION: Lymphoceles are sometimes formed after pelvic lymph node dissection. However, recurrence at lymphoceles has not been reported previously. Here, we report a case of rapid prognosis of the recurrence at a lymphocele after nephroureterectomy. CASE PRESENTATION: A 78-year-old man underwent retroperitoneoscopic radical nephroureterectomy with pelvic lymphadenectomy for left ureteral urothelial carcinoma. The histopathological diagnosis was high-grade invasive urothelial carcinoma with squamous differentiation. Follow-up computed tomography at 3 months postoperatively showed a lymphocele with a small solid component, in the left pelvic region. At 7 months postoperatively, he presented with severe fatigue, and computed tomography showed a solid tumor had replaced the lymphocele. Computed tomography-guided biopsy was performed and histopathological diagnosis was squamous cell carcinoma. CONCLUSION: This report provides support for possible recurrence at the lymphocele after nephroureterectomy. If lymphocele occurs after surgery for malignant disease, it is recommended to follow up with the possibility of recurrence in the lymphatic cysts in mind.