Two Ectopic Liver Lobes Discovered Incidentally at an Autopsy: A Case Report.

The ectopic liver lobe is a rare anomaly and is most frequently reported as a solitary mass. Herein, we report a case of multiple (two) ectopic liver lobes detected at an autopsy. A Japanese man in his 70s died of an infectious disease associated with acquired immunodeficiency syndrome (AIDS). Autopsy revealed the incidental finding of two 1-cm masses, located anterior to the inferior vena cava. Both masses were composed of liver tissue and had internal microscopic structures resembling the porta hepatis, consisting of an outflow bile duct and blood vessels. The outflow bile duct appeared to be continuous with the common bile duct, but the connection point of the outflow vessel was unclear. The liver tissue showed fibrous thickening of the central veins and portal venopathy, including fibrosis in the portal area as well as narrowing and loss of the portal veins. There was no evidence of congestion, fibrosis, biliary stasis, or neoplasm. The incidence of hepatocellular carcinoma is higher in the ectopic liver lobe than in the proper liver, presumably due to the abnormal circulation and bile excretion pathways. The patient also presented with portal venopathy; this suggests the presence of abnormal circulatory dynamics.

A case of Kawasaki disease presenting as sigmoid colitis.

Initial gastrointestinal symptoms might confuse the clinical pictures of some patients with Kawasaki disease (KD) and delay diagnosis and treatment, especially when the patient does not fulfill sufficient diagnostic criteria for KD. Here, we present the case of a 4-year-old boy with KD who complained of severe left abdominal pain and fever alone for the first 6 days. Abdominal ultrasonography showed severe wall thickening localized to the sigmoid colon, and these findings were confirmed by computed tomography and colonoscopy. Microscopic examination of a biopsy specimen revealed non-specific colitis with inflammatory cells in the lamina propria of the sigmoid colon, indicating sigmoid colitis. He subsequently exhibited typical symptoms of KD and was successfully treated with oral administration of aspirin. We suggest that KD should be considered as a differential diagnosis in any child presenting with abdominal symptoms and prolonged fever without definable cause. Abdominal ultrasonography can help evaluate the gastrointestinal complications of KD.

[Rapidly progressive AL amyloidosis in a patient with relapsed multiple myeloma after achieving a complete response to tandem autologous PBSCT].

A 56-year-old male presented with pathological rib fracture and lumbago in 2006. He was diagnosed with multiple myeloma (IgG-lambda type, D&S stage IIIA, ISS 2). He was treated with VAD therapy and tandem auto-PBSCT, and achieved CR in 2007. He was followed without chemotherapy, but relapsed in 2009. He received lenalidomide plus dexamethasone and bortezomib plus dexamethasone and achieved PR which was sustained for 25 months. In 2012, he developed edema of the lower legs and pleural effusion, and was diagnosed as having nephrotic syndrome and heart failure due to AL amyloidosis. He died of renal failure and heart failure 3 months after this diagnosis. Autopsy findings showed amyloid deposition in many organs including the heart, kidneys, liver, spleen, and intestines. Development of rapidly progressive AL amyloidosis is a rare complication of relapse after the achievement of CR, but careful monitoring is needed in patients with multiple myeloma.

A case of perinephric liposarcoma which recurred ten years later from the initial operation.

A 58-year old man was referred to our hospital for treatment of an abdominal mass. As for him, tumor resection with right nephrectomy had been performed ten years ago for a giant well-differentiated perinephric liposarcoma. CT examination showed a huge tumor shadow in the abdominal cavity. Abdominal MRI examination showed a 15 × 8 cm tumor with almost high signal intensity on the T2 weighted images. At laparotomy, a large bulky retroperitoneal tumor pointed out before an operation was found. Surgical extirpation of the tumor was performed. Besides, several tumors of the thumb head size were detected into right retroperitoneal fatty tissue. The right side mesocolon and the tumors were not able to exfoliate, therefore right hemicolectomy was performed. Histological features showed dedifferentiated liposarcoma. The postoperative course was uneventful. But eight months after surgery, he was admitted again for treatment of a 4 × 3 cm retroperitoneal tumor. Extirpation of the tumor was performed. Histological finding of this tumor also showed dedifferentiated liposarcoma. Dedifferentiation, occurring in 15% of the well-differentiated liposarcomas, sometimes may develop later. Long-term detailed follow-up is necessary for well-differentiated liposarcoma.

Gastric cancer with choriocarcinoma and yolk sac tumor components: case report.

Choriocarcinoma- and yolk sac tumor-like differentiation have rarely been reported in gastric cancers. We report a case of gastric adenocarcinoma, concurrently possessing choriocarcinoma and yolk sac tumor components, of a 74-year-old man. A hemorrhagic, 11 × 8 × 3 cm, tumor with ulceration was located in the body and pre-pylorus of the stomach. Histological examination of the resected specimens demonstrated intermingled proliferation of three different components, namely, adenocarcinoma, choriocarcinoma and yolk sac tumor, which were immunoreactive for carcinoembryonic antigen (CEA), beta-subunit of human chorionic gonadotropin (HCG) and alpha-fetoprotein (AFP), respectively. Gastric cancers with germ cell tumor components are uncommon and this is the second reported case of gastric cancer with choriocarcinoma and yolk sac tumor components.

Coexistent poorly-differentiated neuroendocrine cell carcinoma and non-invasive well-differentiated adenocarcinoma in tubulovillous adenoma of the rectum: report of a case.

A 74-years old man was referred to our hospital for treatment of a rectal mass. Colonoscopy revealed villous tumor covering all the lower rectal lumen. Biopsy yielded a diagnosis of adenoma. CT examination showed tumor shadows of the rectum and the liver. Pelvic MRI examination showed a 10.5×8×7 cm tumor with high signal intensity on the T2 weighted images in the rectum. Rectosigmoidectomy with lymph node dissection was performed with the diagnosis of rectal cancer that metastasized to the liver. Histological and immuno- histochemical features showed coexistent poorly-differentiated small cell neuroendocrine cell (NEC) carcinoma and non-invasive well-differentiated adenocarcinoma in tubulovillous adenoma. However the chemotherapy with FOLFOX and Bevacizumab was performed postoperatively, the patient died in cancer 3 months after surgery. Rectal poorly-differentiated NEC carcinomas are thought to be a tumor with a high malignant potential. Recently, the UICC TNM classifications of malignant tumors, 7th edition and the Guidelines for colorectal NEC tumors of European Neuroendocrine Tumor Society have been published. They would be evaluated, and effective multimodal therapy for NEC carcinomas should be established.

Adenoid cystic carcinoma of maxillary sinus with metastatic hepatocellular carcinoma. Case report.

A case of collision tumor in the left maxillary sinus composed of adenoid cystic carcinoma (ACC) and metastatic hepatocellular carcinoma (HCC) is reported. Radiographic examination revealed masses in the liver and bilateral lung metastases. Histologically, proliferation of tumor cells with resemblance to HCC was observed, in addition to the ACC. For this reason, differential diagnosis between a second primary tumor and metastasis was made. The metastatic lesion immunohistochemically showed positivity for hepatocyte antigen (OCH1E5) and protein induced by vitamin K absence or antagonist II (PIVKA-II), sustaining the HCC diagnosis. Primary ACC and metastatic HCC in the maxillary sinus are rare, and this may therefore be the first case of maxillary sinus tumor with both these elements.

Complicated paraneoplastic neurological syndromes: a report of two patients with small cell or non-small cell lung cancer.

Paraneoplastic neurological syndromes are frequently associated in patients with small cell lung cancer (SCLC) and antineuronal antibodies are involved in the autoimmune mechanism. Multiple syndromes are sometimes complicated in a single patient with SCLC. However, little is known about non-SCLC-associated neurological manifestations. We report two patients with complicated paraneoplastic neurological syndromes. Patient 1 showed paraneoplastic limbic encephalitis (PLE), paraneoplastic sensory neuropathy (PSN) and Lambert-Eaton myasthenic syndrome (LEMS) associated with SCLC. Patient 2 developed opsoclonus-ataxia and probable PLE associated with non-SCLC. Analysis of various antineuronal antibodies revealed that anti-Hu and P/Q-type voltage-gated calcium channel (VGCC) antibodies were positive in Patient 1 but any antibodies were not in Patient 2. Brain MRI demonstrated high intensity signals in temporal lobes particularly on fluid-attenuated inversion recovery (FLAIR) or diffusion-weighted images. These findings suggest that complicated paraneoplastic neurological syndromes occur in non-SCLC as well as SCLC and that unidentified antineuronal autoantibodies may underlie the pathophysiology.

The role of mast cells in acute tubulo-interstitial nephritis with uveitis.

UNLABELLED: We describe the clinicopathological characteristics of two patients with acute tubulo-interstitial nephritis with uveitis (TINU) with mast cells infiltrating the interstitium. The pathogenesis of TINU remains unknown, but a T-cell-mediated immune response was suggested to be involved. Recent studies have shown that infiltrating mast cells are closely associated with the development of renal interstitial fibrosis in glomerulonephritis. To address the role of mast cells in the renal interstitial injury in TINU, immunohistochemical studies were performed in renal biopsy sections using anti-human mast cell tryptase antibody specific for mast cells. In addition, we tried to detect CD68-positive macrophages to compare with the localisation of mast cells within the renal interstitium. Mast cells and macrophages could be detected in renal interstitial lesions of both patients. Massive infiltration of macrophages into interstitial lesions was observed, whereas mast cells were detected in a sporadic rather than a clustered manner, and associated with fibrotic lesions. Repeat renal biopsy findings suggested the involvement of these cells in the renal interstitial injury because the number of infiltrating mast cells and macrophages in the interstitium decreased with the improvements in clinical symptoms and pathological lesions. CONCLUSION: The present study showed that mast cells might play an important role in the development of renal interstitial injury in tubulo-interstitial nephritis with uveitis.

Cytological analysis of glycogen-rich carcinoma of the breast: report of two cases.

BACKGROUND: Glycogen-rich carcinoma is a rare special histologic subtype of breast cancer and its incidence is estimated to be 1.4% in breast malignancies. However, its precise characteristics in cytological specimens have not yet been fully clarified. CASE: Fifty-nine-year-old and 53-year-old women underwent fine-needle aspiration biopsy cytology (FNABC) of a breast tumor, confirming malignancy. A mastectomy with axillary dissection was performed. Cytologically, a moderate amount of eosinophilic, finely granular cytoplasm was seen in the majority of the tumor cells, however, foamy and vacuolated cytoplasm was noted in some tumor cells. Histologically, the tumor cells of both cases had clear and granular cytoplasm, which showed a positive reaction with periodic acid-Schiff, eliminated by diastase. CONCLUSION: While clear cytoplasm in the tumor cells in the FNABC seemed to be a pivotal cytological characteristic of glycogen-rich carcinoma, it may not be a major component of cytological specimens. Routine periodic acid-Schiff staining may be required to diagnose glycogen-rich carcinoma in cytological methods.

N-ethyl-N-hydroxyethylnitrosamine (EHEN)-induced renal and hepatocarcinogenesis in the tumor suppressor Tsc2 transgenic rat.

Hereditary renal carcinomas (RCs) develop in Tsc2 gene mutant (Eker) rats around the age of 1 year. We previously reported that Tsc2 mutations were detected in chemically (N-ethyl-N-hydroxyethylnitrosamine (EHEN) and diethylnitrosamine)-induced non-Eker rat RCs, suggesting an involvement of Tsc2 alteration in rat RC development. In this study, we evaluated the effect of extra copies of the Tsc2 gene on renal and hepatocarcinogenesis that was induced by EHEN in vivo. The incidence of RCs in non-transgenic rats (2/17) is slightly higher than in transgenic rats (0/32), although it is statistically not significant. These results suggest the presence of other target RC gene(s) in chemically (EHEN)-induced renal carcinogenesis. We observed no difference in the numbers and areas of the hepatic glutathione S-transferase placental type positive foci.