Pulmonary Involvement of Acute Myeloid Leukemia Mimicking Transfusion-related Acute Lung Injury.

Transfusion-related acute lung injury (TRALI) is defined as a new episode of acute lung injury (ALI) occurring during transfusion or within 6 hours of transfusion completion. A 66-year-old man suffering from acute myeloid leukemia developed acute respiratory distress syndrome after platelet transfusion. TRALI was diagnosed clinically, but an autopsy showed leukemic cells in diffuse pulmonary edema. Anti-human neutrophil antigen (HNA)-3a antibodies were detected in the donor serum, and the HNA-3 genotype of the patient was identified as a/a. This case was considered to represent pulmonary involvement of acute myeloid leukemia, rather than TRALI. A revision of the definition of TRALI accounting for hematological malignancies should therefore be considered.

Pheochromocytoma Multisystem Crisis Behaving Like Interstitial Pneumonia: An Autopsy Case.

Pheochromocytoma multisystem crisis is a rare and life-threatening disease that is associated with numerous symptoms and which is also difficult to diagnose. We herein report an autopsy case of a 61-year-old man who died due to pheochromocytoma multisystem crisis. The patient complained of vomiting and breathlessness. Computed tomography showed a shadow-like region with a similar appearance to interstitial pneumonia. The patient was diagnosed with takotsubo cardiomyopathy induced by severe lung disease based on the results of echocardiography and coronary angiography. The patient was treated for interstitial pneumonia. However, his condition rapidly deteriorated and he died 6 hours after arrival. We were later informed of his extremely high catecholamine serum levels. We found pheochromocytoma with hemorrhage at autopsy. The patient's lungs showed acute passive congestion with edema and extravasation.

[An Autopsy Case of an Elderly Patient with Signet-Ring Cell Carcinoma of the Breast Derived from Lobular Carcinoma].

A 90-year-old woman was referred to our hospital because of abdominal distension and pretibial edema. No tumors were palpable in either breast. Her abdomen was distended and palpitation was noticed. Laboratory examination revealed hypercalcemia and elevated levels of tumor markers(PIVKA- II , CEA, CA19-9, SCC). Contrast-enhanced computed tomography(CT) showed pleural effusion and ascites, an approximately 17-mm high-contrast mass in the D area of the left breast, and osteolysis of the dorsal lumber spine. We initiated treatment for pleural effusion, ascites, and hypercalcemia. However, on her 11th day in the hospital, hematemesis was observed and the patient died. We suspected metastasis of a malignant tumor, but a primary lesion had not been identified; therefore, an autopsy was performed. Autopsy showed a 17-mm mass in the D area of the left breast. Histopathological examination showed the growth of a signet-ring cell tumor that was immunohistochemically ER-positive, PgR-positive, and E-cadherin-negative. The patient was diagnosed with primary signet-ring cell carcinoma of the breast derived from lobular carcinoma. We reported a rare case of primary signet-ring cell carcinoma of the breast with a review of literature.

Co-expression of ERCC1 and Snail is a prognostic but not predictive factor of cisplatin-based neoadjuvant chemotherapy for bladder cancer.

Neoadjuvant chemotherapy (NC) for bladder cancer has been reported to significantly improve the 5-year survival rate. The aim of the present study was to examine the roles of ERCC1 and Snail in determining the response to chemotherapy in bladder cancer treated with NC and radical cystectomy (RC). The expression of the Snail and ERCC1 proteins was determined by immunohistochemical staining of specimens obtained from 58 patients with bladder tumors treated with NC and RC. The correlation between clinical response and the expression of Snail and ERCC1 was investigated. Snail and ERCC1 were co-expressed in 24 (41.4%) of the 58 patients. A marked correlation was found between the expression of Snail and ERCC1 (P=0.001). The co-expression of Snail and ERCC1 was not able to predict pathological complete response (P=0.202). Results of the univariate analysis revealed that the co-expression of Snail and ERCC1 predicted shorter disease-free survival (DFS) and overall survival (OS) than the negative expression of Snail and/or ERCC1. Moreover, the co-expression of ERCC1 and Snail was the only predictive factor for both DFS (P=0.029) and OS (P=0.040). The expression of Snail was correlated with that of ERCC1 and the co-expression of Snail and ERCC1 was the only significant predictive factor of shorter DFS and OS in patients with bladder cancer treated with NC and RC.

Carcinoma ex pleomorphic adenoma of the parotid gland.

BACKGROUND: Carcinoma ex pleomorphic adenoma (CXPA) is a rare aggressive epithelial malignancy arising from a primary or recurrent benign mixed tumor. Only a few case reports describing the radiologic features of CXPA have been published. PURPOSE: To describe and characterize the magnetic resonance (MR) imaging findings of CXPA in the parotid gland and correlate them with pathologic findings. MATERIAL AND METHODS: The MR images of surgically proven CXPA in the parotid gland of five men and five women ranging in age from 28 to 75 years (mean 52 years) were retrospectively reviewed. All MR images were evaluated with emphasis on the size, margin characteristics, extraparotid infiltration, the presence of an encapsulated component, and signal intensity on T2-weighted or short-inversion-time inversion recovery (STIR) images. RESULTS: The average maximal diameter was 4.3 cm. All 10 tumors had ill-defined boundaries, and seven tumors showed extraparotid infiltration, reflecting invasive growth of the malignant component identified on histological examination. Eight tumors had a round encapsulated component and seven of those signal intensities were a mixture of hypo- and hyperintensity on T2-weighted or STIR images. Histological correlation of these components revealed fibrously encapsulated tumors containing hyalinization and myxoid tissue, suggesting degenerated pleomorphic adenoma. Invasive malignant components had non-specific and various signal intensities. CONCLUSION: An invasive parotid mass co-existing with a round encapsulated component is suggestive of carcinoma ex pleomorphic adenoma.

Urine cytology of micropapillary carcinoma of the urinary bladder.

A case of micropapillary carcinoma (MPC) of urinary bladder is presented, in which the urine smear was studied in detail in an attempt to better characterize the cytologic findings of MPC. When the voided urine was examined in low power, cancer cells were scattered in the specimens as compact papillary/spheroidal clusters composed of pleomorphic cancer cells. Solitary carcinoma cells were occasionally observed. High power view of the smear revealed that the papillae/spheroids consisted of high-grade urothelial carcinoma cells. The cancer cells had pleomorphic nuclei with coarsely granular chromatin and thickened, irregular nuclear membrane, and thick cytoplasm. Histologically, the tumor in the resected bladder appeared as small nests with surrounding hallo both in the luminal surface and in the site of wall involvement. These tightly bound papillary/spheroidal clusters comprised of highly atypical cancer cells were the most specific cytologic finding in the urine of MPC, which were considered as a key diagnostic clue of MPC. The background of the urine smear showed numerous granulocytes and bacilli compatible with cystitis, which is a previously known complication of MPC. Differential diagnoses of MPC from those with pertinent cytologic findings such as conventional UC (including glandular differentiation), and primary/secondary adenocarcinoma of urinary bladder are discussed with a brief review of literature.

A patient with rheumatoid arthritis treated with tocilizumab together with lamivudine prophylaxis after remission of infliximab-reactivated hepatitis B.

A 59-year-old woman with rheumatoid arthritis was treated quite successfully with infliximab, but her serum aminotransferase levels were markedly elevated; this was diagnosed as acute exacerbation of hepatitis B and she was treated with lamivudine, and infliximab was discontinued. The rheumatoid arthritis disease activity was uncontrollable after the discontinuation of infliximab, and we therefore initiated tocilizumab treatment (after obtaining the patient's informed consent) together with lamivudine prophylaxis. After tocilizumab administration her rheumatoid arthritis disease activity was significantly attenuated, and the activity has remained low, without re-exacerbation of the hepatitis, for more than 2 years since the initiation of the tocilizumab.

Exfoliated malignant cells at the anastomosis site in colon cancer surgery: the impact of surgical bowel occlusion and intraluminal cleaning.

PURPOSE: Exfoliated malignant cells, present along staple lines of anastomosis, may be responsible for anastomotic recurrence of colon cancer. We aimed to assess the impact of surgical bowel occlusion around the tumor and intraluminal lavage on the presence of exfoliated malignant cells at anastomosis sites in patients with colon cancer. METHODS: In this prospective study, 32 patients with colon cancer, requiring right hemicolectomy between January 2007 and September 2008, were randomly assigned to a control group (no surgical bowel occlusion; 18 patients) and a "no-touch" group that underwent surgical bowel occlusion around the tumor before tumor manipulation (14 patients). The fluid used intraoperatively to irrigate the portion of the bowel clamped distal to the tumor was examined cytologically, and exfoliated cells of cytological classes IV and V were considered malignant. RESULTS: In the control group, 2 (11.1%) and 10 (55.6%) of 18 patients had exfoliated malignant cells at the terminal ileum and distal colon anastomosis sites, respectively; however, only 1 (7.1%) of the 14 patients in the no-touch group had exfoliated malignant cells at both the sites. The frequency of exfoliated malignant cells at the distal colon anastomosis site was significantly lower in the no-touch group (p = 0.0024). No exfoliated malignant cells were found upon saline irrigation of 400 ml or more in either group. CONCLUSION: Measures, such as surgical bowel occlusion around the tumor and intraluminal lavage, can prevent or eliminate exfoliated malignant cells at anastomotic sites in patients with colon cancer.

Clinical usefulness of mitochondrial transcription factor A expression as a predictive marker in colorectal cancer patients treated with FOLFOX.

We previously reported that mitochondrial transcription factor A (mtTFA) preferentially recognizes cisplatin-damaged DNA via physical interaction with p53 and is upregulated by treatment with cisplatin and fluorouracil (5-FU). The aim of the present study was to evaluate whether expression of mtTFA predicts the clinical outcome in patients with metastatic colorectal cancer treated with modified 5-fluorouracil, leucovorin and oxaliplatin 6 (mFOLFOX6). Fifty-nine patients with metastatic lesions from colorectal cancer treated with mFOLFOX6 were included in this study. The subjects consisted of 25 women and 34 men with a median age of 62 years. The patients were treated with oxaliplatin (85 mg/m(2) ) plus leucovorin (200 mg/m(2) ) as a 2-h infusion on day 1, followed by 5-FU (400 mg/m(2) ) bolus and 46-h continuous infusion of 2400 mg/m(2) . The expressions of mtTFA and p53 of resected primary tumors were examined by immunohistochemical analysis. Of the 59 patients, 33 (complete response 1, partial response 32) achieved a confirmed response to therapy. The positive cytoplasmic staining rate for mtTFA was 44.1% and that for p53 was 59.3%, respectively. Strong expression of mtTFA was detected in eight of 33 complete response/partial response (24.2%) and in 18 of 26 SD/PD (69.2%), indicating that mtTFA expression was significantly correlated with response to chemotherapy (P<0.01). Median overall survival was significantly longer in patients without mtTFA expression (P=0.0493). Multivariate analysis revealed that mtTFA expression significantly affected overall survival (hazard ratio 2.10, P=0.036). Immunohistochemical study of mtTFA may be useful for predicting the clinical outcome of metastatic colorectal cancer patients treated with FOLFOX.

Extensive ganglioneuromatosis of gallbladder.

A case of extensive ganglioneuromatosis (GN) of gallbladder is presented. A 38-year-old man presented with a hepatic hilar mass (Ø ~ 48 mm) and gall stones. He had undergone total thyroidectomy for medullary thyroid carcinoma 8 years earlier. The hepatic hilus tumor, which had been clinically suspected to be a lymph node metastasis from the medullary thyroid carcinoma, was found to be pheochromocytoma. The gallbladder, resected with a clinical diagnosis of cholelithiasis, showed extensive transmural GN despite a grossly normal appearance. Taking into account the past history, the patient was diagnosed as having multiple endocrine neoplasia 2b (MEN2b)-associated GN of gallbladder and ectopic pheochromocytoma. As GN of gallbladder in MEN2b has been rarely reported, the histological findings are described in detail and a brief review of literature is carried out.

Sarcomatoid collecting duct carcinoma of kidney diagnosed with urine and renal pelvic lavage cytology.

A case of sarcomatoid collecting duct carcinoma (CDC) of kidney is presented, in which the diagnosis was made cytologically with voided urine and renal pelvis lavage. Cytology of hemorrhagic voided urine revealed highly atypical adenocarcinoma cells with reminiscent ductal structure, which suggested CDC as the most likely diagnosis. Computed tomography and magnetic resonance imaging demonstrated a left renal tumor, and selective lavage of left renal pelvis yielded spindle-shaped, highly atypical cells that indicated sarcomatoid carcinoma. The diagnosis of renal cancer with urine cytology is challenging because of small number of tumor cells in the urine, which are often associated with degeneration. As the urinary cytologic findings of sarcomatoid CDC have not been reported, the characteristic cytologic findings of sarcomatoid CDC are described in detail, and the differential diagnoses with diagnostic pitfalls were discussed.

[A case of retroperitoneal solitary fibrous tumor associated with hypoglycemia].

Solitary fibrous tumor is an uncommon mesenchymal lesion, seldom associated with hypoglycemia and rarely presents as a retroperitoneal tumor. We present a case in a 56-year-old male patient whose initial symptom was unconsciousness due to hypoglycemia (blood glucose 16 mg/dl). Computed tomography indicated a mass (about 20 x 15 x 15 cm) in the retroperitoneum. Only surgical treatment was performed and the patient has been without hypoglycemia and recurrence for 12 months after surgery. This case had "patternless pattern" characterized by a haphazard, storiform arrangement of short spindle or ovoid cells, and "hemangiopericytoma-like appearance" with prominent vascularity by thin-walled vessels, as expressed CD34 immunohistochemically. Pathohistological examination revealed increased cellularity in the tumor tissues as well as tissues with atypical nuclei of the tumor cells with some cell division. Western immunoblotting and immunohistochemistry showed that tumor produced big IGF-II, leading to hypoglycemia. Due to these findings, it was diagnosed as malignant solitary fibrous tumor associated with non-islet cell tumor hypoglycemia (NICTH). This case is the second malignant solitary fibrous tumor in the retroperitoneum with hypoglycemia in the English literature.

[Female urethral adenocarcinoma with urinary retention: a case report].

A 57-year-old woman presented with urinary retention. Computed tomography revealed a pelvic mass. Vaginal examination revealed an elastic-hard mass beneath the anterior vaginal wall. Intravenous pyelography demonstrated the filling defect of bladder. Magnetic resonance imaging revealed the mass that had invaded bladder neck at dorsal side of urethra. Pathological examination of transvaginal needle biopsy suggested well-differentiated adenocarcinoma. Total cystectomy and ileal conduct formation was performed. Pathological diagnosis was urethral adenocarcinoma. She had local recurrence at perineum at seven months after the operation, and tumor resection was performed. Five months after the operation, she had no evidence of recurrence.

[Urothelial carcinoma of ureter with neuroendocrine differentiation: a case report].

A 73-year-old woman complained of gross hematuria. Urine cytology showed small cell carcinoma (SmCC) cells mixed with urothelial carcinoma (UC) cells. Cystoscopy revealed a bladder tumor around the right ureteral orifice. Abdominal CT demonstrated severe right hydronephrosis. Pelvic MRI showed a mass (approximately phi5 cm) in the urinary bladder wall that protruded into the lumen. TUR biopsy of the bladder tumor was SmCC. With laparoscopic nephrectomy/open ureterectomy, distal end (approximately 10 cm) of the right ureter was tumorous and obstructed. The tumor invaded into the urinary bladder/peritoneum and was unresectable. The tumor of lower-end of ureter tumor was SmCC, showing neuroendocrine differentiation such as pseudo-rosette, positive Grimelius stain, and chromogranin A/NCAM/synaptophysin immunostaining. Proximal to this tumor, non-invasive UC spread superficially over entire length of the ureter to the renal pelvis. Abrupt transition from invasive SmCC to non-invasive UC was observed in the middle of the ureter. Post-operative adjuvant chemotherapy was discouraged due to senile dementia of the patient. The patient died nine months after the operation because of systemic progression of cancer.

[Carcinosarcoma of the prostate: a case report].

A 63-year-old Japanese man with a 1-month history of dyschezia and dysuria was referred to our hospital. Transrectal ultrasonography and magnetic resonance imaging demonstrated a mass involving the prostate, measuring about 6.5 cm. The serum prostate specific antigen was 20.3 ng/ml. By the transperineal needle biopsy, it was suspected to be fibrosarcoma. Total pelvic exenteration was performed. Histopathological examination of the tumor revealed carcinosarcoma of the prostate. One month after the operation local recurrence was manifested by perineal pain. The recurrent tumor grew rapidly with severe pain. The patient's condition deteriorated, and finally he died of the disease nine months after the operation. Carcinosarcoma of the prostate is a rare neoplasm and very aggressive. There is no established protocol for its treatment and its prognosis is usually poor. This is the 49th case reported in the literature.

[Combined small-cell carcinoma/adenocarcinoma of prostate: report of two cases].

We report two cases of combined small-cell carcinoma (SCC) and adenocarcinoma of prostate. Case 1 was a 76-year-old man with loss of appetite and body weight and neck lymphadenopathies. Whole body computed tomography (CT) revealed prostatic swelling, pancreatic mass, para-aortic lymphadenopathies, and multiple lung nodules. Elevation of tumor markers (prostate specific antigen [PSA, 1,760 ng/ml] and neuron-specific enolase [NSE, 88 ng/ml]) was noted. Needle biopsy of the prostate demonstrated both SCC and adenocarcinoma. Only within the part of SCC, were neuroendocrine (NE) markers (chromogranin A [CgA], NCAM, and synaptophysin [SNP]) expressed. Maximum androgen blockade (MAB) resulted in a decrease of PSA (5.13 ng/ml) but an increase of NSE (810 ng/ml). Cytotoxic chemotherapy was not possible because of his poor performance state and renal dysfunction. The patient died three months after the diagnosis. Case 2 was a 69-year-old male with dysuria. The symptom and elevated serum PSA (23.1 ng/ml) prompted prostatic needle biopsy, which demonstrated combined SCC/adenocarcinoma. NE markers (CgA and SNP) were weakly expressed in the part of SCC. Serum NSE was 6.9 ng/ml. After MAB, serum PSA dropped to the normal range (0.192 ng/ml) and the effect of MAB was judged as complete response (CR). The patient has been alive for 15 months with no signs of relapse. Treatment of combined SCC and adenocarcinoma of prostate poses a dilemma. In Case 1, MAB was effective for adenocarcinoma but not for SCC. The opposite situation would be expected with systemic chemotherapy. However, the histologically similar Case 2 achieved CR with MAB alone. Much remains to be elucidated to better manage combined SCC/adenocarcinoma of prostate.

[Prostatic carcinoma that arose with hearing loss: a case report].

A 69-year-old male with tinnitus, vertigo, and progressive hearing loss of left ear was admitted to our hospital. Head magnetic resonance imaging and computed tomography (CT) revealed swelling of multiple neck lymph nodes (LNs) invading the skull base, which involved left mastoid sinus/the eighth cranial nerve. Biopsy of the cervical LN demonstrated small-cell carcinoma (SCC). Whole body CT showed systemic lymphadenopathies (subclavian, para-aortic, and bilateral iliac LNs) and prostatic swelling with multiple pelvic masses. Needle biopsy of the prostate revealed SCC (Gleason score: 5+ 5). Immunohistochemically, neuron-specific enolase (NSE) and NCAM were detected in <10% and -100% of cancer cells, respectively. Despite SCC histology, prostate-specific antigen (PSA) and androgen receptor (AR) were also expressed in -20% and -70% of tumor cells, respectively. Serum PSA and NSE were 464 ng/ml and 12 ng/ml, respectively. After maximum androgen blockade (MAB) with leuprorelin/bicalutamide, the patient showed recovery of hearing loss, regression of cervical LNs (partial response), and decline of serum markers (PSA 7.38 ng/ml and NSE 3.7 ng/ml, respectively). As re-increase of PSA was observed after ten months, MAB menu was changed to leuprorelin/fultamide. Another four months later, the treatment was changed to docetaxel/ estramustine due to the appearance of systemic bone pain and recurrence of LN metastases. He is alive (39 months after diagnosis) with cancer. Widespread metastases at the time of diagnosis were compatible with SCC. However, this case was AR-positive and responded to androgen ablation, at least temporarily. Even though the initial symptoms are atypical for a prostatic carcinoma, SCC of prostate needs to be included as a rare differential diagnosis.

[Primary adenocarcinoma of renal pelvis and ureter suspected as metastatic tumor: a case report].

A 58-year-old man, who had undergone sigmoidectomy for sigmoid colon adenocarcinoma 3 years earlier, was referred to our clinic because of left ureteral tumor incidentally found by computed tomography (CT). Under the diagnosis of left ureteral carcinoma, retroperitoneoscopic left nephroureterectomy was performed. Pathological examination revealed adenocarcinoma of the left renal pelvis and ureter. Subtype of the adenocarcinoma was 'enteric type'. Five months later, urine cytology was positive and multiple non-papillary tumors were found on cystoscopy. By the transurethral resection of the tumors, bladder tumors appeared to be adenocarcinoma. Carefully considering the pathological findings and clinical course, we concluded that the tumor was not metastatic but primary adenocarcinoma followed by intravesical recurrence.

[Prostate small cell carcinoma: report of two cases that differed in treatment responsiveness].

Case 1 : A 76-year-old man with a chief complaint of dysuria had an elevated prostate specific antigen (PSA) level of 24.9. He underwent a transperineal needle biopsy of the prostate, and the histopathological diagnosis was prostatic small cell carcinoma. The cancer was clinically diagnosed as T3bN1M1 with multiple lung metastases. He started receiving hormonal therapy. After three months of hormonal therapy, the multiple lung metastases disappeared. Thereafter, the serum PSA level and the tumor volume increased and he died 12 months from the start of therapy. Case 2: A 79-year-old man was referred to our hospital with a chief complaint of dysuria. The serum level of PSA was elevated to 10.4. Transperineal prostate biopsy revealed prostatic small cell carcinoma. The cancer was clinically diagnosed as T3bN1M1, and hormonal therapy was started. Subsequently, although his serum PSA level declined, his condition worsened rapidly and he died five months after the start of therapy.