Uroplakin-III to distinguish primary vulvar Paget disease from Paget disease secondary to urothelial carcinoma

Paget disease of the vulva can be mimicked by several disease entities histopathologically, but most of these entities can be clinically distinguished from vulvar Paget disease. However, vulvar Paget disease is in itself a heterogeneous group of epithelial neoplasms that can be similar both clinically and histopathologically. The subtypes of vulvar Paget disease include primary Paget disease arising from a pluripotent stem cell within the epithelium of the vulva, and secondary Paget disease of the vulva. Secondary vulvar Paget disease results from spread of an internal malignancy, most commonly from an anorectal adenocarcinoma or urothelial carcinoma of the bladder or urethra, to the vulvar epithelium. We have recently proposed that these lesions be classified as primary (of cutaneous origin) or secondary (of extracutaneous origin). These subtypes can present similarly as eczematoid skin lesions and may appear similar on routine hematoxylin and eosin-stained slides. Immunohistochemical studies can help differentiate between them. Our current study includes 17 patients with a pathologic diagnosis of vulvar Paget disease. We performed a panel of immunohistochemical stains, including cytokeratin (CK) 7 and 20, carcinoembryonic antigen (CEA), gross cystic disease fluid protein-15 (GCDFP-15), and uroplakin-III (UP-III). Of these 17 patients, 14 (80%) had primary intraepithelial cutaneous Paget disease, 13 without invasion and 1 with associated invasion. Three patients had urothelial carcinoma with spread to the vulva, manifesting as secondary vulvar Paget disease. Immunohistochemically, primary vulvar Paget disease is immunoreactive for CK 7 and GCDFP-15, but uncommonly for CK 20. Vulvar Paget disease secondary to anorectal carcinoma demonstrates CK 20 immunoreactivity but is usually nonreactive for CK 7 and consistently nonimmunoreactive for GCDFP-15. Vulvar Paget disease secondary to urothelial carcinoma is immunoreactive for CK 7 and CK 20 but nonimmunoreactive for GCDFP-15. In addition, we propose the use of a new, commercially available antibody, UP-III, which is specific for urothelium and, in our experience, is immunoreactive in secondary vulvar Paget disease of urothelial origin. The distinction between these 3 types of Paget and Paget-like lesions is essential in that the specific diagnosis has a significant influence on current treatment. The difference in surgical approach to the subtypes of vulvar Paget disease justifies classifying them into distinct lesions, which may be aided by the use of immunohistochemistry, including UP-III.     

浸润性乳腺Paget病3例并文献复习

目的：探讨浸润性乳腺Paget病(invasive mammary Paget disease,invMPD)的临床病理特征。方法：回顾性分析3例浸润性乳腺Paget病的临床资料和病理资料并复习文献。结果：3例患者均有乳头糜烂,1例伴有乳头溃疡,1例同侧乳腺左侧外上象限浸润性癌,1例伴有同侧乳腺高级别导管内癌。组织学表现为具有典型的乳腺Paget病的特征,可见小的浸润灶突破基底膜,浸润真皮浅层。免疫组织化学示肿瘤细胞均表达ER、CK7、EMA;2例表达PR,2例HER-2(3+),1例HER-2(2+),Ki67从40%~60%;S-100、P63均阴性。结论：浸润性乳腺Paget病是一种罕见的肿瘤,与非浸润性乳腺Paget病(non-invasive mammary Paget disease,non-invMPD)有相似的预后。     

Inverted variant of urothelial carcinoma of the urinary bladder: a report of three cases and a proposal for a new clinicopathologic entity

Inverted urothelial carcinoma (UC) without papillary areas is very rare; only 31 cases of three papers have been reported. The author herein reports three additional cases, and proposes the term “inverted variant” (IV) of UC. The materials were 3 cases of IV of UC, 5 cases of inverted papilloma (IP), and two cases of nested variant (NV) of UC. The three cases of IV of UC consisted of 56-year-old woman, 63-year-old man, and 78-year-old man. Presenting symptoms were hematuria in all cases. The cystoscopic findings were elevated tumors without papillary proliferations in all cases. The treatment was transurethral tumor resection (TUR-BT) in all cases. The sizes was 0.6 cm, 0.5 cm, and 3 cm. Microscopically, IV of UC showed inverted growth of atypical cells without papillary proliferations. Compared to IP, the inverted growth pattern was similar, but cytological atypia and thick trabeculae were noted in IV of UP while they were absent in IP. Compared to NV of UC, the growth pattern is different; NV of UC showed nested and vague tubular pattern. The cellular atypia is more pronounced in IV of UC than NV of UC. Immunohistochemically, p53 expression was seen in all the cases of IV of UC and in all the cases of NV of UC, while p53 expression was negative in all the cases of IP. Ki-67 labeling index was 25, 30 and 40% in IV of UC, 15 and 30% in NV of UC, and 3, 5, 6, 7, 9% in IP. Invasive features were seen in 1 case of IV of UC and 2 cases of NV of UC. In all cases of IV of UC, IP, and NV of UC, the TUR-BT, but one case of IV of UC, showed no recurrence after TUR-BT, while one case of IV of UC showed a recurrence. In conclusion, the IV and UC were structurally and cytologically very different from the NV of UC. The IV of UC was structurally similar to IP, but cellular atypia and thickened trabeculae were seen in IV and UC. p53 expression and Ki-67 labeling status were entirely different between in IV of UC and IP. The author proposes the term of IV of UC as a new clinicopathological entity.     

Pulmonary adenocarcinoma: review of 106 cases and proposed new classification.

The gross and microscopic appearances of 106 resected pulmonary adenocarcinomas were reviewed and correlated with postoperative survival. Instead of using an established classification based on histological pattern, the tumours were categorised by cellular morphology and site as either parenchymal adenocarcinoma (67%), bronchial adenocarcinoma (13%), or adenocarcinoma of uncertain origin (20%). Despite their pleomorphic appearance parenchymal adenocarcinomas should be regarded as a single entity, derived from multipotential cells of the distal airway; bronchial adenocarcinomas were generally, but not invariably, associated with short postoperative survival; those tumours that could not be reclassified on histological grounds were large adenocarcinomas consisting mainly of mucus cells. Tumours of this type carry a poor prognosis.     

Restrictive dermopathy: a report of three cases.

We report three infants with a rare syndrome of restrictive dermopathy, in which rigidity of the skin at birth is associated with characteristic facial anomalies, generalised arthrogryposis, bony abnormalities, and lung hypoplasia. The skin has a distinctive pathology with compaction of the dermal collagen and fibrosis of the subcutaneous tissue. The inheritance is likely to be autosomal recessive and the condition appears to be fatal in the early neonatal period.     

Fibrosing cholestatic hepatitis: a report of three cases

Fibrosing cholestatic hepatitis is an aggressive and usually fatal form of viral hepatitis in immunosuppressed patients. We report three cases of fibrosing cholestatic hepatitis in various clinical situations. Case 1 was a 50-year-old man who underwent a liver transplant for hepatitis B virus (HBV)-associated liver cirrhosis. Two and a half years after the transplant, he complained of fever and jaundice, and liver enzymes were slightly elevated. Serum HBsAg was positive. Case 2 was a 30-year-old man in an immunosuppressed state after chemotherapy for acute lymphoblastic leukemia. He was a HBV carrier. Liver enzymes and total bilirubin were markedly elevated. Case 3 was a 50-year-old man who underwent renal transplantation as a known HBV carrier. One year after the transplant, jaundice developed abruptly, but liver enzymes were not significantly elevated. Microscopically lobules were markedly disarrayed, showing ballooning degeneration of hepatocytes, prominent pericellular fibrosis, and marked canalicular or intracytoplasmic cholestasis. Portal inflammation was mild, but interphase activity was definite and cholangiolar proliferation was prominent. Hepatocytes were diffusely positive for HBsAg and HBcAg in various patterns. Patients died of liver failure within 1 to 3 months after liver biopsy in spite of anti-viral treatment.     

Giant cell fibroblastoma: a report of three cases

Three cases of giant cell fibroblastoma are presented. All three patients were boys younger than four years of age. The neoplasms involved the subcutaneous tissue of the back, perineum, and shoulder, respectively. The tumors were characterized histologically by a mixture of spindle cells and multinucleated giant cells in a myxoid or collagenous background and by the presence of irregular, sinusoidal spaces. All three patients had local recurrence, but all were tumor-free at latest follow-up.     

Disseminated disease caused by Mycobacterium simiae in AIDS patients: a report of three cases

Disseminated disease caused by Mycobacterium simiae, a slowly growing nontuberculous mycobacterium, has been rarely reported in the literature. We report on three AIDS patients who were found to suffer from mycobacteraemia caused by M. simiae in a rural tertiary-care hospital in central India.     

致死性侏儒-附三例报告

目的研究致死性侏儒的临床、影像学、致病基因和病理学表现。方法复习1438例尸检中诊断为致死性侏儒的3例作回顾性研究。结果致死性侏儒胎儿长骨极短，可有不同程度的弯曲，四肢软组织堆积，胸腔狭小、肋骨短，心胸比例大于正常，腹部膨隆，头颅大、前额突，塌鼻。结论致死性侏儒是一种最常见的致死性短肢畸形，由纤维母细胞生长因子受体3（FGFR3）基因突变所致，具有明确的临床、影像学和病理学特征。     

Cytological features of lipoblastoma: a report of three cases

Lipoblastoma is a rare benign neoplasm occurring mostly in children under the age of three. Accurate preoperative diagnosis is mandatory for planning the treatment. The main aim of this study is to establish the cytological features of lipoblastoma and to answer the question: “Is cytological diagnosis of lipoblastoma reliable?” Preoperative fine‐needle aspiration biopsy (FNAB) and tissue sections of three children treated for lipoblastoma at the Division of Pediatrics, University Medical Center, Ljubljana, Slovenia, in the period from 1997 to 2004 were reexamined. The Giemsa‐ and Papanicolaou‐stained FNAB smears were moderately or poorly cellular and contained lipocytes, lipoblasts, and spindle cells in various proportions. The tumor cells were in clusters and tissue fragments or as single cells. Thin branching capillaries were observed in most of the clusters and tissue fragments. In the background, abundant myxoid extracellular material and naked oval nuclei were present. In the first case, the cytological diagnosis was benign soft‐tissue tumor, in the second the diagnosis was not conclusive and the last case was correctly diagnosed as lipoblastoma. In the differential diagnosis of the second case, both lipoblastoma and liposarcoma were considered cytologically as well as histologically. At 7‐yr follow‐up, there is no evidence of the disease. Thus, we conclude that lipoblastoma with typical cytological features could be accurately diagnosed by FNAB. However, tumors containing numerous lipoblasts could pose a diagnostic problem. Diagn. Cytopathol. 2005;33:195–200. © 2005 Wiley‐Liss, Inc.     

外阴Paget's病放射治疗1例并文献复习

外阴Paget's病是一种罕见疾病,其临床症状无特异,易被误诊、漏诊.对外阴Paget's病的诊断应结合患者症状、影像学表现及病理检查等.手术治疗是首选治疗方式,无法手术的患者放疗是其重要的治疗手段.     

Jeune syndrome and liver disease: report of three cases treated with ursodeoxycholic acid

Evidence for a bipolar disorder (BPD) susceptibility locus on chromosome 22q11 has been provided in several studies. One candidate gene that maps to this region is the G-protein alpha subunit gene Galphaz (GNAZ). We have identified a common silent polymorphism in GNAZ exon 2 by single strand conformation polymorphism analysis. The frequency of this polymorphism was determined in a control population (n=84) and in patients with BPD (n=88). The data showed a statistical trend toward a difference in the distribution of alleles in patients with BPD compared with control subjects (chi square=3.2, 1 df, P=0.073, two-tailed). No significant difference was detected when the GNAZ polymorphism was analyzed in control subjects and schizophrenia patients (n=63, P=0.92). These data continue to provide some support for a BPD susceptibility gene on 22q11, possibly in linkage disequilibrium with the GNAZ 309 polymorphism.     

Pregnancy in Sheehan's syndrome: a report of three cases

Postpartum pituitary necrosis (Sheehan's syndrome) is a relatively common clinical disorder in developing and underdeveloped areas of the world. Sheehan's syndrome has a spectrum of presentations. Spontaneous pregnancy in such patients is a rare occurrence. Three patients with clinical and hormonal evidence of postpartum pituitary necrosis conceived spontaneously during the follow up period. Pregnancy though rare may occur either due to sparing or recovery of gonadotroph function in such patients.     

IgA deficiency: a report of three cases from Thailand

Selective IgA deficiency has been reported to be the most common primary immunodeficiency disease in Western countries. A markedly lower frequency of this condition has been reported in the Japanese population. While most of the IgA deficient cases are healthy, some patients develop significant recurrent sinopulmonary infections, allergic disorders and autoimmune diseases. Herein, we report three cases of IgA deficiency among Thai patients, all of whom suffered from chronic sinopulmonary infections. Two of the three patients had absolute IgA deficiency while the third had a partial IgA deficiency. The associated conditions found in these three patients were deficiencies of an IgG subclass, allergic rhinitis and lupus nephritis. The youngest child (5 years old boy with lupus nephritis) expired from Pneumocystis carrinii pneumonia complicated with adult respiratory distress syndrome.     

Wilms' tumour in adults: a report of three cases

Wilms' tumour is the most common pediatric renal neoplasm. Its occurrence in adults is rare, less than 1% of all nephroblastomas have been reported after the age of 15 years of age. We report 3 cases of Wilms' tumour in adults, their clinico-pathological profile and review the literature including the cases reported from India. Patients were young adults, their age ranging from 25 to 32 years. The largest tumour dimension was between 11 cm to 17 cm. Two cases were blastema predominant while the third case had typical triphasic histology. All cases were stage II (National Wilms' Tumour Study), favourable histology. Although Wilms' tumour in adults is morphologically similar to its childhood counterpart, it often responds poorly to the combination chemotherapy. The criteria for its diagnosis are strict and should be adhered to for its correct identification.