A Rare Case of Pulmonary Tumor Thrombotic Microangiopathy Associated with Micropapillary Urothelial Carcinoma of the Urinary Bladder: An Autopsy Case.

A 70-year-old woman was hospitalized with dyspnea. A transthoracic echocardiogram indicated an elevated systolic pulmonary artery pressure, and the cytology specimens obtained using a pulmonary artery catheter confirmed adenocarcinoma metastasis. Diffusion-weighted whole-body imaging with background body signal suppression (DWIBS) detected high-signal-intensity lesions in the urinary bladder. The patient died of respiratory failure and a postmortem examination was performed. Tumor cells in the bladder were immunohistochemically positive for GATA3, indicating micropapillary urothelial carcinoma, which is a rare variant of urothelial carcinoma and considered an adenocarcinoma subtype. This case is the first autopsy case of pulmonary tumor thrombotic microangiopathy (PTTM) associated with micropapillary urothelial carcinoma of the urinary bladder.

Multiple Esophageal Squamous Papillomas.

We report a rare case of multiple esophageal squamous papillomas (ESPs). A 42-year-old man underwent a medical examination, and abnormalities of multiple elevated lesions were noted using a esophagogastroduodenoscope. He underwent upper gastrointestinal tract radiography with an orally ingested barium sulfate and gastrointestinal endoscopy, which revealed multiple elevated lesions in the esophagus, predominantly on the distal esophagus. We performed an endoscopic esophageal mucosal resection using a cap-fitted esophagogastroduodenoscope to obtain sufficient specimens. Based on the pathological findings, we diagnosed multiple ESPs. Although single ESPs or a few ESPs in a patient are often encountered, multiple ESP cases are rare.

Crystal-storing histiocytosis associated with marginal-zone lymphoma.

We report a rare case of crystal-storing histiocytosis (CSH) associated with marginal-zone lymphoma. A 91-year-old woman with a history of breast cancer presented with masses of the posterior neck, right breast, and left upper arm. An enlarging mass of the neck was removed and was histologically diagnosed as CSH, associated with marginal-zone lymphoma. Masses in the breast and upper arm were evaluated by needle biopsy and revealed as CSH. CSH is a rare condition characterized by the intrahistiocytic accumulation of crystallized immunoglobulins, and is associated with disorders in which monoclonal immunoglobulins are expressed. To the best of our knowledge, there are few previous descriptions of CSH which include the imaging features of this disease. In the present case, the masses showed hypoechogenicity with internal patchy hyperechoic areas on ultrasonography, and an iso-signal to slight hyperintensity compared with muscle on T2-weighted magnetic resonance imaging. We report the imaging findings in CSH and discuss their diagnostic implications.

Utility of preoperative visualization for intrapulmonary sequestration in video-assisted thoracoscopic surgery.

We herein report the case of a 59-year-old male suffering from severe cough. The patient had previously experienced several episodes of pneumonia beginning in childhood. A three dimensional multidetector computed tomography (3D-MDCT) scan revealed pulmonary sequestration with a left gastric artery blood supply, and clearly revealed the other pulmonary vessels. The patient underwent video-assisted thoracoscopic surgery, in which the anomalous tissue was resected safely. Preoperative visualization of the vessels may be helpful for the safe treatment of a pulmonary sequestration, and the VATS approach is a suitable operation under corrective surgical planning.

Angiomyofibroblastoma-like tumor (cellular angiofibroma) in the male inguinal region.

Angiomyofibroblastoma-like tumor is a rare mesenchymal tumor involving the male genital tract. We report a case of an angiomyofibroblastoma-like tumor that arose in the subcutaneous tissue of the left inguinal region in a 50-year-old man. Ultrasonography of the region demonstrated a well-circumscribed subcutaneous mass. Intralesional fat was revealed on magnetic resonance images. Although these imaging features are nonspecific, radiological findings enable considering the diagnosis of angiomyofibroblastoma-like tumor.

Mechanisms of inactivation of the p16INK4a gene in leiomyosarcoma of soft tissue: decreased p16 expression correlates with promoter methylation and poor prognosis.

The p16INK4a tumour suppressor gene, encoding p16 protein, plays a crucial role in regulation of the G1 cell-cycle phase. To investigate the potential role of p16 in soft tissue leiomyosarcoma (LMS), an immunohistochemical analysis was performed of 77 LMSs for p16 expression. Decreased expression of the p16 protein was identified in 25 of 77 LMSs (32%). Decreased expression of p16 correlated significantly with large tumour size (p=0.0038). In a univariate analysis, large tumour size and decreased expression of p16 were statistically significant adverse prognostic factors (p=0.025 and p=0.0021, respectively). In a multivariate analysis including conventional clinicopathological parameters, decreased expression of p16 protein was revealed as the only independent unfavourable prognostic factor (p=0.012). To elucidate the mechanisms of inactivation of the p16INK4a gene, 49 LMSs for which genomic DNA was available were examined; analysis for homozygous deletion, mutation, and promoter hypermethylation was conducted using differential PCR, PCR-SSCP, and methylation-specific PCR, respectively. Promoter hypermethylation was detected in 11 of 49 LMS cases (22%); homozygous deletion was detected in 3 of 49 cases (6%); and mutation was not recognized in any of the cases studied. Eight of 15 cases (53%) with decreased expression of p16 protein revealed methylation of the p16INK4a gene promoter. Promoter hypermethylation correlated closely with decreased expression and poor prognosis (p=0.0014 and p=0.0088, respectively). These results suggest that decreased expression of p16 protein can be considered as an independent reliable prognostic parameter in patients with soft tissue LMS. Furthermore, promoter methylation was more frequent than either homozygous deletion or mutation in this tumour, and promoter methylation was also shown to have a strong association with inactivation of the p16INK4a gene.

Cytogenetic and clinicopathological analysis of soft-tissue leiomyosarcomas.

To identify a characteristic cytogenetic aberration and cytogenetic-morphological correlation in soft-tissue leiomyosarcomas, a karyotypic and clinicopathological analysis of 15 cases of leiomyosarcoma was performed. The histological type was classical in nine cases, pleomorphic in three cases and myxoid in three cases. The histological grade was 1 in three cases, 2 in 10 cases and 3 in two cases. Nine of 15 tumors displayed an abnormal karyotype, whereas the other six tumors displayed a normal karyotype. The relative consistency of involvement of 3p, 3q, 6q, 7p, 7q, 9p, 10p, 11p, 11q, 12p, 16q, 17p and 19q was recognized, although characteristic chromosomal rearrangements were not detected. All six tumors that had a normal karyotype were of the classical type, whereas those displaying an abnormal karyotype contained another morphological type along with the classical type. The results of the present study suggest that chromosomal aberrations contribute to morphological changes in soft-tissue leiomyosarcomas.