Acute onset collagenous colitis associated with protein-losing enteropathy.

Collagenous colitis is a cause of chronic diarrhea. We report an atypical case of collagenous colitis, presenting with an acute onset, and associated with protein-losing enteropathy. An 82-year-old woman was admitted with a 1 week history of nausea, appetite loss, and diarrhea. Serum albumin level was low. Protein leakage from the small intestine was found by a Technetium-99m human serum albumin scintigraphy. We diagnosed the patient with collagenous colitis from pathology findings of multiple biopsies taken from the colon. This case implies that collagenous colitis should be considered in acute watery diarrhea, and that it can cause protein-losing enteropathy.

Romanowsky staining using liquid-based cytology: A pilot study using Cytolyt(®)/HESPANDER(®) processing solution for ThinPrep(®) preparations.

BACKGROUND: Liquid-based cytology (LBC) has been used to prepare and examine many types of samples. However, the use of Romanowsky stains for LBC has not yet been evaluated. Herein, we report a technique for the use of the Romanowsky May-Grünwald-Giemsa (MGG) stain using a ThinPrep(®) preparation technique (MGG-LBC). METHODS: KPL-1 breast cancer cells at a density of 1.25 × 10(5)/20 ml were compared in conventional smear and MGG-LBC preparations. Cell size, nucleus/cytoplasm (N/C) ratio, and morphological findings were investigated. Clinical samples including voided urine and pleural effusion were also examined in MGG-LBC preparations. RESULTS: Cellularity appeared lower with MGG-LBC compared with Papanicolaou-stained smears using the ThinPrep(®) method, though the cell size and N/C ratio showed similar tendencies. Reactive mesothelial cells, normal urothelial cells, urothelial carcinoma cells, crystals, and bacteria were all clear enough for diagnostic purposes after MGG-LBC. CONCLUSION: Romanowsky staining is necessary for the cytodiagnosis of some conditions. MGG-LBC may contribute to the cytodiagnostic results using LBC preparations.

Diagnosis of intraocular lesions using vitreous humor and intraocular perfusion fluid cytology: Experience with 83 Cases.

OBJECTIVE: The objective of the study was to evaluate the use of vitreous humor and/or intraocular perfusion fluid (IPF) from pars plana vitrectomy as a diagnostic and therapeutic procedure for intraocular diseases. METHODS: The cytologic findings with respect to the clinical data, the anatomical findings, and the final diagnosis in 83 cases that underwent intraocular cytologic examinations at the Kansai Medical University Takii Hospital were evaluated. For cytologic examination, the Papanicolaou stain, Giemsa stain, and in some cases, molecular biology and immunocytochemical techniques were used. RESULTS: Most of the clinical diagnoses were uveitis or endophthalmitis. Sixty-eight cases (81.9%) were negative on cytodiagnosis, while 15 cases (18.9%) were positive or suspicious for malignancy. Negative cases included infections and intraocular sarcoidosis (IOS), and all of the positive or suspicious cases were intraocular lymphomas. Some of these latter cases were also diagnosed using immunocytochemical staining or molecular biological procedures as ancillary techniques, performed using vitreous body cytology from IPF. CONCLUSIONS: An early diagnosis and treatment of intraocular diseases is necessary to maintain an acceptable degree of quality of life. For an accurate diagnosis, it is necessary to understand the anatomy of the eye. Giemsa staining is recommended in addition to Papanicolaou staining for cytologic diagnostic evaluation of intraocular diseases. Furthermore, for the diagnosis of clonality in intraocular lymphomas, it is often necessary to use ancillary molecular biological procedures, using vitreous fluid.

Horrifying Basal cell carcinoma: cytological, immunohistochemical, and ultrastructural findings.

Basal cell carcinoma (BCC) is a slow-growing and frequently occurring tumor of the eyelids. Among BCC cases, there is a subtype of aggressive cases called horrifying BCC (HBCC). There are also rare BCC cases that show neuroendocrine differentiation. Here, we describe a case of HBCC with neuroendocrine differentiation. The patient, a 41-year-old woman, presented with abnormal left eye tearing and left cheek pain. On computed tomography imaging, a tumor that extended to the left orbit was detected in the left cheek. On cytological examination of fine-needle aspiration (FNA) samples, the tumor cells were observed as sheet-like clusters and single bare nuclei with a clear background; peripheral palisading was not clearly seen. On examination of the biopsy specimen taken after FNA, the tumor was found to be composed of cancer cell nests with scattered peripheral palisading in the dermis. Immunohistochemically, the tumor cells were positive for cytokeratin (CK) 7 and CD56 and were negative for CK20, synaptophysin, and chromogranin A. Membrane-bound dense-core granules were detected on ultrastructural study. A HBCC case with neuroendocrine differentiation has not been previously reported. The correlation between the presence of neuroendocrine differentiation in HBCC and patient prognosis should be further studied.

Multifocal adenomatous oncocytic hyperplasia of the parotid gland.

Multifocal adenomatous oncocytic hyperplasia (MAOH) is a non-neoplastic lesion that is classified as oncocytosis. MAOH is a rare entity of the parotid gland and accounts for approximately 0.1% of salivary gland lesions. Here, we report a case of MAOH of the parotid gland. The patient was a 71-year-old woman who presented with discomfort at the left side of her neck. Fine-needle aspiration cytology of the parotid gland revealed a loose sheet-like cluster of round to polygonal cells with granular cytoplasm against a hemorrhagic background. The cells had round to oval, centrally located nuclei with granular chromatin and without distinct nucleoli. Histologically, the lesion was formed of many variable-sized nodules, comprising oncocyte-like cells with small round nuclei and eosinophilic granular cytoplasm that was positive for mitochondrial antibodies. The diagnosis of MAOH is difficult to make by cytology alone, because the findings overlap with those of other oncocytic lesions. In particular, the cytological findings of MAOH have not been sufficiently reported to date. A correlation of cytology and histology was expected.

Estrogen receptor- and progesterone receptor-positive diffuse sclerosing variant of papillary thyroid carcinoma: a case report.

The diffuse sclerosing variant of papillary thyroid carcinoma (DSV-PTC) is a relatively rare tumor. We herein report the case of young woman with DSV-PTC who developed cervical lymph node recurrence 7 years after the initial surgery. A 15-year-old female patient with no medical or family history of thyroid tumors developed a thyroid neoplasm in the right lobe. Right thyroidectomy and regional lymphadenectomy were performed, and the tumor was diagnosed as DSV-PTC. She was followed up as an outpatient. Seven years after the surgery, cervical lymph node recurrence developed. On microscopic examination, the thyroid tumor showed a papillary growth pattern with numerous psammoma bodies and distinct fibrosis. Immunohistochemically, the tumor cells were estrogen receptor and progesterone receptor positive with reduced membranous expression of E-cadherin and were intermingled with S-100-positive dendritic/Langerhans cells. DSV-PTC is characterized by a strong tendency for invasion and metastasis. Thus, accurate diagnosis is clinically important, and a morphological and immunohistochemical understanding of DSV-PTC is necessary.

Latent adrenal Ewing sarcoma family of tumors: A case report.

Ewing sarcoma family of tumors (ESFT) is derived from the neural crest, which originates from basal embryo cells in the primitive neural tube. ESFT often arises at the bone, chest wall, and soft tissues of the thoracic region. However, ESFT that arises from the adrenal gland is much rarer and it is usually revealed by clinical symptoms. We report an autopsy case of suicidal hanging, in which adrenal ESFT was incidentally revealed. To our knowledge, this is the first case of latent ESFT arising from the adrenal gland. Autopsy can sometimes reveal latent disease. Some of these latent diseases are very rare and we would not be able to detect them without a complete autopsy. As forensic pathologists, we should attempt to perform a complete autopsy and report new discoveries for the development of medicine.

Two cases of malignant peritoneal mesothelioma without asbestos exposure: cytologic and immunohistochemical features.

Approximately half a century has passed since asbestos was first reported to be the main cause of malignant mesothelioma; yet the incidence of this disease continues to increase worldwide. Twenty percent of cases occur without prior asbestos exposure, and in these patients, malignant peritoneal mesothelioma is more common than malignant pleural mesothelioma. Here, we report the cytomorphologic and immunohistochemical features of 2 cases of malignant peritoneal mesothelioma where there was no history of asbestos exposure. Ascitic cytology showed that most cells were isolated and that clusters were rarely observed, but the findings were consistent with malignant mesothelioma in both cases. Immunohistochemical analysis for epithelial membrane antigen, calretinin, vimentin, ß-catenin, melan-A, glucose transporter-1, cytokeratin CAM5.2, Wilms tumor antigen-1, D2-40, CD146, progesterone receptor, estrogen receptor, and cytokeratin 5/6 was indicative of malignant mesothelioma. In malignant mesothelioma without prior asbestos exposure, the etiology and prognostic significance is still unclear. Further study is needed to clarify this point.

Retrospective cytological study of intraocular lymphoma using vitreous and intraocular perfusion fluid.

Intraocular lymphoma (IOL) is an extremely rare tumor. We carried out a retrospective cytopathological study with vitreous and intraocular perfusion fluid obtained on conducting a pars plana vitrectomy in 18 cases of IOL. In the 18 cases, nine were patients of Kansai Medical University Takii Hospital from 1991 to 2007, and the other nine had already been reported by other hospitals. Most patients were male, and the average age at onset was 60.4-year-old. The main symptoms were vitreous opacity, amblyopia, and blurred vision. Cases of primary intraocular lymphoma numbered 8/15 (53%), while cases of infiltration of malignant lymphoma from the brain numbered 2/15 (13%). Although IOL contains various subtypes of lymphoma, the most frequent subtype is diffuse large B-cell type lymphoma. It has been reported that making a definite diagnosis of IOL is difficult because the clinical symptoms and examinations are similar to chronic uveitis, and so IOL is called "masquerade syndrome." Recently, serological and molecular pathological studies have been carried out in addition to morphological examination. However, a cytological diagnosis based on the clinical background and/or image findings is important for the diagnosis of IOL, because of the volume limit of the vitreous fluid and difficulty of obtaining specimens from the inside of the eyes.

Utility of immunohistochemical analysis of KAI1, epithelial-specific antigen, and epithelial-related antigen for distinction of chromophobe renal cell carcinoma, an eosinophilic variant from renal oncocytoma.

Distinction of renal oncocytoma (RO) from chromophobe renal cell carcinoma (ChRCC) is important because their clinical behavior is different. As part of a search for the best available immunohistochemical markers to distinguish ChRCC from RO, we investigated the immunohistochemical profiles of these tumors. We selected 30 renal tumors consisting of ChRCC, typical variant (n = 14), ChRCC, eosinophilic variant (n = 6), and RO (n = 10). Their expression of cytokeratin (CK) 7, KAI1, epithelial-specific antigen (ESA), epithelial-related antigen (ERA), Claudin- 7, and Claudin-8 was studied using an autostainer. Immunoreactivity was assessed based on a combined score of the extent and intensity of staining. Compared to RO, a significantly higher percentage of the total ChRCCs stained positive for CK7 (85% vs. 10%, respectively), KAI1 (90% vs. 10%), ESA (95% vs. 10%), ERA (95% vs. 10%), and Claudin-7 (95% vs. 20%) (P < 0.001). Additionally, there was a significant difference between the percentage of ChRCC eosinophilic variant (ChRCC-E) and RO that stained positive for KAI1 (100% vs. 10%, respectively), ESA (83% vs. 10%), and ERA (83% vs. 10%) (P < 0.001). We recommend immunohistochemical analysis of KAI1, ESA, and ERA to distinguish ChRCC-E from RO.

Diagnostic utility of vitreous humor fluid cytology for intraocular sarcoidosis: a clinicopathologic study of 7 cases.

Sarcoidosis is an enigmatic multisystem sarcoid disease occurring mainly in the lungs, skin, and eyes. Sarcoidosis of the eyes (intraocular sarcoidosis) has been proposed as one of the causes of uveitis. We carried out a clinicopathological study involving seven patients with intraocular sarcoidosis based on vitreous humor fluid cytology. In the seven patients, the sex ratio was 4:3, and the average age at onset was 65.6-years-old. Vitreous opacity was noted in all patients and snowball-like vitreous opacity in 85.7%. Nerve papilloedema was noted in 42.9%. Diagnostic vitrectomy and adrenocortical hormone therapy were performed in all patients. As a result, the symptoms were improved. Vitreous perfusion fluid cytology revealed multinucleate giant cells in 85.7% and lymphocytes and epithelioid cells in all cases. Cytological diagnosis is not included in the current criteria for sarcoidosis, but its value in the diagnosis of intraocular sarcoidosis was suggested based in present study.

Case report of death from falling: Did heart tumor cause syncope?

A healthy man in his 30s was working on the balustrade of stairs on the second floor. He suddenly fell downstairs without saying anything. On emergency hospitalization, chest echogram showed left hemothorax. Cardiac echogram showed a floating mass from the mitral valve in the left ventricle and severe mitral regurgitation. Surgery for hemothorax and pulmonary contusion was immediately undertaken. However, bleeding from pulmonary contusion could not be controlled and he underwent cardiopulmonary arrest. Autopsy showed a white, elastic, pendulous mass in the left atrium and a white mass in the lower lobe of the left lung. Tumor histology showed a reticular pattern, Schiller-Duval bodies, eosinophilic hyaline globules, and positive staining for α-fetoprotein. We diagnosed primary lung yolk sac tumor with metastatic intracardiac yolk sac tumor, a rare and highly malignant germ cell tumor. It usually arises in the ovaries and testes, and intracardiac yolk sac tumor is rare. Intracavitary tumors induce obstruction of inflow into and outflow from the ventricular cavity. The most common clinical presentation is dyspnea and syncope. In the present case, metastatic cardiac yolk sac tumor might have disturbed cardiac outflow and affected hemodynamics, probably causing syncope. Unfortunately, he was in a high place at that time and fell to receive pulmonary contusion that led to death. Autopsy may sometimes reveal latent diseases which might be related to the cause of death. We should perform autopsy thoroughly to diagnose not only the cause of death but also the factors leading to death.

Well-differentiated liposarcoma, an atypical lipomatous tumor, of the mesentery: a case report and review of the literature.

Mesenteric liposarcoma is a rare neoplasm. Here, we report the case of a 73-year-old Japanese man with a well-differentiated (WD) liposarcoma of the mesentery. Due to rapid growth of the abdominal mass and abdominal insufficiency, a tumorectomy was performed. The excised tumor was 12.4 × 9.6 cm in size and weighed 548 g. Cut sections showed a lobulated yellow and/or grayish-colored appearance. The histological features were predominantly those of the sclerotic and lipoma-like variants of WD liposarcoma. The cytoplasm of most spindle cells was diffusely immunoreactive for CD34, while fat cells were positive for S-100 protein. Some spindle cell nuclei were positive for CDK4, and a few were positive for MDM2. The average Ki-67 proliferation index in tumor cells was 10%, and androgen receptor expression was detected in tumor cell nuclei. The present case and 11 cases identified from a literature search were reviewed. The WD mesenteric liposarcomas developed in patients in the fourth to seventh decades of life (mean age 57.9 years). The patients consisted of 7 men and 5 women. All tumors were larger than 10 cm in diameter at the time of surgery. Complete resection might be the only curative therapy for WD liposarcomas of the mesentery, but long-term follow-up is needed because of the possibility of a local recurrence of the tumor.

Endometrial clear cell adenocarcinoma diagnosed by endometrial cytological examination: two cases report.

In contrast to endometrioid endometrial adenocarcinoma (EA), endometrial clear cell adenocarcinoma (ECCA) is rare and shows a clinically aggressive potential, resulting in a poor prognosis. Therefore, the early diagnosis of ECCA is crucial for the treatment of patients bearing the tumor. Here, we report two cases of ECCA diagnosed by cytological examination. Case 1 was a 67-year-old female, while case 2 was a 68-year-old female. In both cases, tumors of the corpus uteri were identified, and they were diagnosed as ECCA on cytological examination with specimens derived from endometrial brushing. The diagnosis was confirmed by the histological examination of the surgical specimens.

A case of splenic low-grade mucinous cystadenocarcinoma resulting in pseudomyxoma peritonei.

Primary splenic mucinous cystadenocarcinoma (MCCa) is extremely rare, and only six cases appear to have been reported previously. We present herein a case of primary splenic MCCa resulting in pseudomyxoma peritonei (PMP). A 66-year-old Japanese woman presented to a hospital with a chief complaint of upper abdominal pain and a 7-year history of splenic cyst. Cyst rupture was noted on computed tomography, and splenectomy was performed. The abdominal cavity was filled with a large amount of gelatinous ascites, with the appearance of PMP. On the cut surface, multiple cysts containing mucinous material were found within and outside the spleen. Microscopically, splenic parenchyma was occupied by large mucinous pools focally lined with mucinous epithelial cells and mesothelial cell-like cells, which were considered benign. Outside the spleen, a low-grade MCCa component was found. No ectopic pancreatic or intestinal tissue was identified. Although most PMP cases are known to be caused by low-grade mucinous appendiceal tumor, the present case represents the first report of a splenic MCCa resulting in PMP.

Impact of obesity on IgA nephropathy: comparative ultrastructural study between obese and non-obese patients.

BACKGROUND: The pathological role of obesity in the progression of glomerular lesions has rarely been studied in primary glomerular diseases. The purpose of this study is to investigate the influence of non-diabetic obesity on clinicopathological findings in IgA nephropathy. METHODS: 74 patients with biopsy-proven IgA nephropathy were retrospectively divided into two groups according to the criteria for obesity in Japan: non-obese group (group N: n = 50) with BMI <25 kg/m(2), and obese group (group O: n = 24) with BMI > or =25 kg/m(2). Clinical and pathological data at the time of renal biopsy were analyzed. Moreover, the outcome of proteinuria in patients treated with angiotensin-converting enzyme inhibitors (ACE-I) or angiotensin II receptor blockers (ARB) was evaluated in different groups after a 1-year follow-up. RESULTS: Urinary protein excretion was significantly greater in the obese group compared to normal-weight patients (p < 0.05). There was no significant difference in the prevalence of hypertension and hyperlipidemia. By light microscopy, the obese group showed significantly larger glomerular size (p < 0.0001). On the other hand, the severity of mesangial matrix expansion and crescent formation revealed no difference between the two groups. By electron microscopy, glomerular basement membrane (GBM) thickness was significantly increased in obese patients (p < 0.001). Among 61 patients who were followed up for 1 year in our institute, 15 patients were treated with ACE-I or ARB without steroids. ACE-I or ARB treatment without steroids tended to reduce proteinuria in the obese patients, but this change did not achieve statistical significance. CONCLUSIONS: In IgA nephropathy, obesity induces not only glomerular enlargement but also ultrastructural modification of GBM, which would contribute to increase proteinuria.

Glomeruloid hemangioma.

A 64-year-old Japanese woman suffering from idiopathic thrombocytopenic purpura was treated with prednisolone. During the course of steroid withdrawal she developed parotid gland enlargement and cervical lymph node swelling with multiple dome-shaped red papules on her trunk and upper limbs. On admission the patient was found to have numbness of her lower limbs (polyneuropathy), lymph node swelling (organomegaly), high glucose level (endocrinopathy), Bence-Jones protein-kappa in the urine (M protein) and skin with hyperpigmentation, hypertrichosis and multiple glomeruloid hemangiomas (skin abnormalities), indicating polyneuropathy-organomegaly-endocrinopathy-M-protein-skin abnormality (POEMS) syndrome. The patient was also found to have peripheral edema, ascites, and pleural effusion. The glomeruloid hemangiomas had intravascular capillary growth, which was composed of conglomerates of capillaries resulting in structures resembling renal glomeruli. Cells within the capillary loops were lined by endothelial cells with scant cytoplasm (CD31(+)/CD34(+)/CD68(-)/CD105(+)/UEA-1(+)) while the outer surfaces of the loops were covered by either swollen endothelial cells containing PAS- and immunoglobulin-positive eosinophilic hyaline globules (CD31(+)/CD34(-)/CD68(-/+)/CD105(-)/UEA-1(-)) or cells without globules. These two phenotypically different endothelial cells were separated by alpha-smooth muscle actin-positive pericytes. Pericytes and endothelial cells covering the outer surface of the loops were bordered by basement membrane. Biopsy of parotid gland and lymph node indicated Sjögren's syndrome and Castleman's disease of a hyaline-vascular type, respectively. Resumed prednisolone therapy has been successful, and the patient was left with minimal residual symptoms. Glomeruloid hemangioma is a specific marker of POEMS syndrome and is related to Castleman's disease. Idiopathic thrombocytopenic purpura and Sjögren's syndrome may also be related.

Nephronophthisis complicated with hepatic fibrosis: an autopsy case with rupture of the splenic artery after renal transplantation.

Nephronophthisis (NPHP) is a disease characterized by a genetic cause of chronic renal failure in children and adolescents, complicated with several extra-renal manifestations such as retinal defect and/or liver fibrosis. Although it is difficult to establish the correct diagnosis, mutations in six genes (NPHP 1-6) have recently been identified. Here we report the case of a 25-year-old male with NPHP with congenital hepatic fibrosis. He showed microscopic hematuria and moderate proteinuria at 20 years. Renal biopsy revealed severe interstitial fibrosis, diffuse tubular atrophy and microcysts at this time with chronic kidney disease stage III (Cr 2.43 mg/dl). C3c was positive in glomeruli in direct immunofluorescent study. Although his mother belongs to a family with polycystic kidney disease, he did not have a novel genetic background of Arg585Cys mutation in exon 8 of the PKD1 gene. Magnetic resonance angiography (MRA) showed typical portal hypertension with spleno-renal shunt caused by biopsy-proven liver fibrosis. Thus, we diagnosed him as having undetermined renal cystic or tubulo-interstitial disease complicated with membranoproliferative glomerulonephritis (MPGN). Renal transplantation was performed in January 2005 after 2 years of dialysis therapy. He was transported to our emergency room because of severe abdominal pain in December 2005. A computed tomographic scan showed massive ascites, which were caused by rupture of the splenic artery. Despite full intensive care including intraluminal coiling of the ruptured aneurysm and extensive blood transfusion, we failed to rescue him on the next day. The autopsy findings revealed severe atrophy of the bilateral kidney with multiple cysts along the cortico-medullary border. Obvious portal hypertension, resulting from congenital hepatic fibrosis, could account for the rupture of the splenic artery with aneurysm formation under pressure/volume overload. This is the first report of a NPHP patient with the complication of hepatic fibrosis emerging from an ADPKD family. As it remains elusive on the phenotype-genotype of the Japanese NPHP population, a registration system of cystic disease of the kidney is required.

Esophageal involvement in microscopic polyangiitis: a case report and review of literature.

A 72-year-old man with cough and sputum showed esophageal wall thickening and pneumonia in chest computed tomography (CT) scan. Following endoscopy, we diagnosed reflux esophagitis and subscribed proton pump inhibitor. The esophageal lesion, however, was intractable. We diagnosed microscopic polyangiitis (MPA) because of vasculitis symptoms, cytoplasmic antineutrophil cytoplasmic antibodies (cANCA) in blood and no granulomatous change in the esophagus. We adopted pulse therapy of cyclophosphamide and oral prednisolone; the symptoms and esophageal lesion were markedly improved. We concluded that the esophageal lesion was an aspect of MPA. To our knowledge, this is the first report of esophageal involvement in MPA.