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Aims Dermatopathic reaction is a histopathological finding of lymph nodes that usually occurs in patients with inflammatory pruritic cutaneous lesions. However, it is sometimes seen in patients with cutaneous T cell lymphoma. Adult T cell leukaemia/lymphoma (ATLL) is a T cell malignancy caused by infection with human T cell leukaemia virus type I (HTLV-1), which is frequently accompanied by cutaneous lesions. However, the detailed clinicopathological characteristics of the dermatopathic reaction of lymph nodes in ATLL patients and HTLV-1 carriers, addressed in this study, remains to be clarified. Methods and results We retrospectively analysed 18 nodal lesions with dermatopathic reaction in HTLV-1 carriers. Axillary and inguinal lymph nodes were the primary affected tissues. Three cases with atypical lymphoid cell infiltration were defined as ATLL with dermatopathic reaction (ATLL-D), showing an abnormal T cell immunophenotype and T cell monoclonality. Two of the three ATLL-D patients died 14 and 7 months after diagnosis (the third case had a very short follow-up). The other 15 patients were indistinguishable from reactive lesions and were defined as HTLV-1-associated lymphadenitis with dermatopathic reaction (HAL-D). They showed an indolent clinical course, with only one case eventually transforming to aggressive disease. Conclusions Lymph node lesions accompanied by dermatopathic reaction in HTLV1 carriers represent a spectrum that includes reactive and neoplastic conditions. HAL-D should be distinguished from ATLL-D, especially to avoid overtreatment.

A 3-month-old boy developed small papules on his trunk. After the papules increased in number, the patient was diagnosed with Langerhans cell histiocytosis based on the pathological findings. He was referred to our department for further examination. Upon initial examination, the papules and nodules were scattered on his back, abdomen and lumbar region. Because he did not present with any organ involvement except the skin, he was diagnosed with single-system and skin-limited Langerhans cell histiocytosis. Skin rashes were treated with a topical steroid and started regressing 3 months after onset. All papules disappeared 6 months after onset. In this boy, the Langerhans cell histiocytosis tumor cells expressed phosphorylated extracellular signal-regulated kinases. In Langerhans cell histiocytosis, BRAF V600E and other genes are known to mutate to act as driver mutations in stem cells of the myeloid dendritic cell lineage. Consequently, extracellular signal-regulated kinases are continuously activated, which contributes to Langerhans cell histiocytosis carcinogenesis.

Local estrogen metabolism and its sensitivities in the skin have been also suggested to contribute to skin homeostasis in addition to age- and/or gender-dependent circulating estrogen, even though their local mechanisms have been largely unknown. To characterize their potential correlations, age- and gender-dependencies were evaluated focusing on 5 pivotal estrogen-metabolizing enzymes including aromatase, estrogen sulfotransferase, steroid sulfatase, and 17β-hydroxysteroid dehydrogenases and estrogen receptors (ERα and ERβ) using immunohistochemistry of 100 human skin specimens. When their epidermal expression levels were compared among 7 age groups, ranging from the teens to the seventies, the highest expression in the teens group and the lowest expression in the seventies group were found in the expression of aromatase and ERβ, respectively, while no significant differences between the male and the female groups were found in the immunoreactivities of our interested proteins. Our results suggest that age-related differences in aromatase and ERβ expressions impact epidermal homeostasis.

Malignant fibrous histiocytoma (MFH) is the most common fibroblastic tumor, but its cutaneous metastasis, especially in-transit metastasis, is extremely rare. We describe the case of a 30-year-old Japanese man with a recurrent MFH on the scalp accompanied by in-transit metastasis, which had been treated as a benign skin tumor 8 years before. The main bulk of the recurrent tumor was located in the dermis, but the metastatic tumor was mainly located in the subcutis. Generally, atypical fibroxanthoma, also known as cutaneous MFH, is rarely metastasized and presents a benign clinical course. Since there is a great difference between the prognosis of MFH and atypical fibroxanthoma, precise diagnosis of the primary tumor is essential., Article, Case reports in dermatology.3:164-169(2011)

目的 : 東北大学病院病理部における上皮型と二相型悪性中皮腫 (malignant mesothelioma, 以下 MM) 16 例の, 体腔液細胞診の正診率は 7/16 (43.8%) であった. 今回, MM の診断率の向上を目的とし, MM の診断に至らなかった 9 例の要因について細胞学的に検討を行った.方法 : MM と診断した 7 例, MM の診断に至らなかった 9 例 (陰性 4 例, 疑陽性 4 例, 腺癌 1 例) の体腔液細胞診と, 65 例の良性体腔液中の反応性中皮細胞 (reactive mesothelial cell, 以下 RM) を対象とした. 各症例について, 細胞の出現形式, 細胞集塊の形状, 細胞と核の性状および面積, 多核細胞の割合について比較検討した.成績 : 陰性とした 4 例中 3 例の MM 細胞は少なく, ほとんどは孤立性に出現していた. また, 細胞面積は RM (243.6μm2) に比し同程度から小さかった. 疑陽性および腺癌と判定した症例では, 多数の MM 細胞が孤立性や重積性集塊で出現していたが, 細胞面積は 5 例中 4 例が RM より小さかった.結論 : MM では出現細胞量が少なく, 腫瘍細胞が小型な症例では, 陰性または疑陽性判定の要因となることが示唆された. MM の診断および RM との鑑別には, 重積性集塊, 重厚な細胞質, 腫大した好酸性の核小体, 多核細胞の割合 (10%以上) が重要な細胞所見と考えられた.

Aim: We evaluated the diagnostic efficacy of transpapillary intraductal ultrasonography before biliary drainage (IDUS-BD) and transpapillary biopsy (TPB) for the assessment of the longitudinal extent of bile duct cancer. Methods: Between November 1999 and January 2005, we performed IDUS-BD and TPB preoperatively in 27 patients with carcinoma of the extrahepatic bile duct. Following IDUS-BD, TPB was performed under fluoroscopic guidance immediately after endoscopic sphincterotomy. The diagnostic efficacy of IDUS-BD and TPB for the longitudinal extent of the cancer and the complications which accompanied the procedure were evaluated. Results: The overall success rate of sampling and the diagnostic accuracy of bile duct cancer by TPB were 85.3% (192/225) and 85% (23/27), respectively. The sensitivity, specificity and accuracy of the assessment of the longitudinal extent of cancer on the hepatic and duodenal sides by IDUS-BD were 82%, 70%, 78% and 85%, 43%, 70%, respectively. Those by a combination of IDUS-BD and TPB were 88%, 80%, 85% and 77%, 86%, 80%, respectively. Overestimation of the longitudinal extent of BD cancer by IDUS-BD was mainly due to inflammation and obscure images, especially resulting from collapse of the bile duct on the duodenal side of the tumor, and was corrected by TPB in four of five patients. No serious complications occurred following the combination of IDUS-BD and TPB. Conclusions: TPB is useful for preoperative histological diagnosis of bile duct cancer. The combination of IDUS-BD and TPB is practical for evaluation of its longitudinal extent; basically, IDUS-BD is sufficient on the hepatic side of the tumor, but concomitant TPB is recommended on the duodenal side.

Immunohistochemistry is widely used for pathological diagnosis of breast lesions. Other than hormone receptors and HER2/neu analysis for primary breast carcinomas, several markers may be useful for differential diagnoses, although in limited situations. To decide the malignant potential of intraductal proliferative lesions, analysis for the staining pattern of cytokeratins may be a good reference. Most ductal carcinoma in situ cases are diffusely positive for luminal cell markers (CK8, CK18, CK19), but negative for basal cell markers (CK5/6 and CK14). However, usual ductal hyperplasia may show the mosaic staining patterns for any of these markers, which may indicate a heterogeneous cell population in benign lesions. Myoepithelial markers (alpha-SMA, myosin, calponin, p63, CD10) are almost consistently positive for benign papillomas but they do not completely distinguish intraductal papillary carcinomas. Preservation of myoepithelial layer is the diagnostic key when looking at benign sclerosing lesions, including carcinoma with pseudoinvasive structures. E-cadherin is mostly positive for ductal carcinomas but negative for lobular carcinomas. Some of the lobular carcinomas are positive for 34betaE12, but they are consistently negative for CK5/6. Comparison with histopathological findings of hematoxylin and eosin is essential to make proper diagnosis in the individual case.

A 55-year-old male patient with hepatocellular carcinoma underwent transcatheter arterial embolization (TAE). He became febrile and experienced pain at the right hypochondrial region 323 days later, which led to the discovery of a liver abscess that fistulated into the duodenal bulb. There have been no reports on the fistulation of liver abscesses into the digestive system following TAE. Rhodococcus equi was isolated as a causative agent, which distinguished the case further.

肺癌骨転移の診断は一般に骨シンチグラムを代表とする画像診断で行われているが定量性, 偽陽性, 経費および簡便性などの問題があった.近年, 新しい骨代謝マーカーが開発されたので, これらの肺癌骨転移に対する有用性を検討した.ICTPは骨転移のある肺癌症例で9.0±5.2ng/mlであり良性肺疾患3.7±1.1ng/mlおよび骨転移のない肺癌症例4.6±1.6ng/mlに比較して有意に高値であった (p

Importance Dermatofibrosarcoma protuberans (DFSP) is a rare skin cancer that develops in the deep dermis to subcutaneous adipose tissues. A COL1A1-PDGFB gene fusion, leading to the constitutive expression of PDGFB, is the tumorigenic mechanism in most DFSP cases. Objectives To evaluate the specificity of PDGFB expression as a diagnostic marker of DFSP and to determine whether other pathomechanisms (ie, gene fusions) exist in patients with DFSP without the COL1A1-PDGFB fusion gene. Design, Setting, and Participants All patients with DFSP registered in the pathologic database of the University of the Ryukyus from January 1, 1997, through December 31, 2013, and Gunma University from January 1, 1996, through December 31, 2011, were included in this analysis. Samples were obtained from 30 patients presenting with DFSP tumors. We examined the clinicopathologic characteristics and the expression of PDGFB, PDGFRβ, PDGFRα, CD34, nestin, factor XIIIa, fibronectin, α–smooth muscle actin, S-100 protein, and Ki-67 in 30 DFSP cases and 48 non-DFSP mesenchymal tumor cases by immunohistochemical analysis. We then analyzed tumor tissues for the presence of the COL1A1-PDGFB fusion gene. We also tested whether other genes enriched in fibroblasts formed fusion products with PDGFB by reverse transcription–polymerase chain reaction analysis, using gene-specific primers. Main Outcomes and Measures We aimed to analyze tumor tissues for the presence of the COL1A1-PDGFB fusion gene to investigate expression of PDGFB in DFSP tumors. Results PDGFB expression was detected in 28 (93%) of 30 patients with DFSP. PDGFB was not homogenously expressed in DFSP tumor cells, whereas CD34 and nestin were often expressed throughout the tumor mass. In 1 DFSP tumor, the COL1A1-PDGFB fusion gene was not detected even though PDGFB was expressed. We identified a novel COL1A2-PDGFB fusion gene in this tumor. Conclusions and Relevance Our findings indicate that PDGFB protein is expressed in most DFSP tumors and may be a useful diagnostic tool when used in conjunction with CD34 and nestin expression analysis. These PDGFB expression data, in addition to our discovery of a novel PDGF fusion gene, strongly support the concept that DFSP is a PDGFB-dependent tumor type.

Sex steroids have been known to play important roles in the homeostasis of human skin, but little is known about their biosyntheses in that tissue. In this study, we characterized the correlation between the concentrations of sex steroids and the expression levels of the factors involved in their synthesis or metabolism in human skin. The expression levels of aromatase (ARO) and steroidogenic acute regulatory protein (StAR) were positively correlated with estrogens and testosterone concentrations, respectively. We demonstrated that estrogen synthesis was markedly decreased by ARO inhibitor and that skins with higher ARO expression had thicker elastic fibers than those with lower ARO expression. While pregnenolone and testosterone concentrations were increased by cholesterol administration to epidermal keratinocytes. Scalp skin with higher StAR expression was cleared to have significantly fewer hair follicles than that with lower expression. Our results suggest that the status of ARO and StAR contribute to estrogen synthesis in situ, especially for the regulation of elastic fiber formation, and to testosterone synthesis, which may be associated with hair growth, respectively.

Sex steroids have been postulated to influence pathophysiology of human skin through various skin appendages. The presence of sex steroid receptors has been also reported in adnexal tumors but its details still remained unknown. Therefore, in this study, we immunolocalized sex steroid receptor protein (estrogen receptor (ER)alpha, ERbeta, progesterone receptor (PR)A, PRB and androgen receptor (AR)) in 23 cases of non-pathological skin (male: 10, female: 13) and in 50 cases of skin adnexal tumors (male 24, female 26; 38 benign and 12 malignant). ERalpha immunoreactivity was detected exclusively in basal cells of sebaceous glands of non-pathological skin. AR and PRB immunoreactivity was detected in both differentiated and basal cells of sebaceous gland. AR and ERbeta immunoreactivity was also detected in sebaceous and eccrine sweat glands but not in outer root sheath of hair follicles. In sebaceous gland neoplasms, the number of ERalpha positive cases was significantly lower in skin appendage neoplasms than non-pathological skin. ERbeta immunoreactivity was not detected in any of sebaceous gland neoplasms examined. There were no significant differences in PRA, PRB and AR immunoreactivity between non-pathological sebaceous gland and its neoplasm. In sweat gland neoplasms, the number of AR positive cases was significantly lower in benign neoplasms than their non-pathological counterpart. Therefore sex steroids are considered to play important roles in regulation of non-pathological skin appendage function and pathogenesis and/or development of its neoplasm. In addition, the status of the great majority of sex steroid hormone receptors was maintained throughout the process of neoplastic transformation of skin appendages, except for AR and ERalpha in sweat and sebaceous gland neoplasms.

It is difficult to heal ulcers during continuous administration of nonsteroidal autiinflammatory drugs (NSAIDs). We retrospectively studied 136 rheumatoid arthritis patients who were taking NSAIDs at least 3 months, and who could be followed within 12 weeks of being diagnosed with an ulcer using endoscopy. Among patients who had ulcers in the antrum (A region), healing rates were 46.7% (21/45), 46.7% (21/45), and 73.3% (11/15) in the famotidine (F) group, lansoprazole (L) group, and misoprostol (MP) group, respectively; the rate was higher in the MP group than in the F and L groups. On the other hand, for ulcers in the body (M region) there were no differences among the groups; healing rates were 94.1% (16/17), 86.7% (13/15), and 100% (1/1), respectively. To study the background regarding A region ulcers and cardia/body (C/M) region ulcers, we studied acid output and Helicobacterpylori (HP)-positive rates. There were no differences in acid output between the two groups. In the NSAID group, the HP-positive rate was significantly lower among A region ulcers (48.3%) than in C/M region ulcers (100%). A large-scale, multicenter study that also considers the role of HP is needed to establish the best treatment of NSAIDS-induced ulcers.

© 2009 The Authors. doi: 10.2340/00015555-0599 Journal Compilation © 2009 Acta Dermato-Venereologica. ISSN 0001-5555 Sir, Epithelioid sarcoma is a soft tissue tumour of uncertain origin. Epithelioid cells with eosinophilic cytoplasm are present together with spindle cells. On the other hand, epithelioid sarcoma-like haemangioendothelioma (ESH) is a rare, low-grade, potentially malignant vascular neoplasm first described by Billings et al. in 2003 (1). Until now only 8 cases have been reported (1, 2). Because of their clinical and histological similarities, it may be misdiagnosed initially as epithelioid sarcoma. However, ES-H is generally indolent compared with epithelioid sarcoma. We describe here the clinical and histopathological features of a case of ES-H that developed in a middle-aged Japanese man.

Recently, sentinel lymph node (SLN) biopsy has been employed to avoid unnecessary lymph node dissection, because SLN negativity for carcinoma metastases may imply an extremely low possibility of non-SLN involvement. Pathological evaluation is essential, but standardized procedures have not yet been determined. Intraoperative consultation, either by frozen section (multiple slices are desirable) or touch imprint cytology, are usually very useful. Their accuracy, however, is variable and depends on the procedures used, but specificity is characteristically 100%, and the missed metastatic focus is always quite minute. After fixation, multiple sections, immuno-histochemistry, and their combination will be able to detect small metastatic foci more frequently. The clinical significance of small or submicro- or occult metastases have not yet been clarified, and further investigations are needed. If the SLN is positive for carcinoma metastases, both the procedure for detection and the size of the metastatic focus should be clarified on the pathological reports.