Dermatopathic lymphadenitis. The spectrum and significance of its morphologic features

The lymph nodes from 50 axillary node dissections were evaluated by light microscopy and immunohistochemistry for the presence of dermatopathic lymphadenitis-like histologic features. On the basis of hematoxylin-eosin-stained sections alone, a histologic continuum was observed ranging from minimal paracortical changes to fully developed dermatopathic lymphadenitis. Twenty-three lymph node biopsies previously interpreted as diagnostic of dermatopathic lymphadenitis were reviewed. Only nine of these showed the fully developed dermatopathic lymphadenitis. Only one of these patients had a dermatitis. Our conclusions are that the presence of dermatopathic lymphadenitis does not mean that the patient has concomitant skin disease, and that axillary lymph nodes from patients without skin disease manifest a spectrum of dermatopathic lymphadenitis-like histologic features. Dermatopathic lymphadenitis may represent one end of a normally occurring histologic spectrum that may be found in the absence of a dermatitis.     

Sialoblastoma and epithelial tumors in children: their morphologic spectrum and distribution by age.

This commentary addresses the histologic spectrum of salivary gland neoplasms in children with emphasis on perinatal tumors. Histopathologically, perinatal tumors fall into four categories: 1) histologically benign with adult counterpart, 2) hamartomas 3) embryomas-sialoblastomas, and 4) histologically and biologically malignant adult analogue tumors. Although the criteria to serrate benign from malignant sialoblastomas are not well-established, the following histologic features would favor an aggressive clinical course: perineural and/or vascular spaces invasion, necrosis, and a cytologic atypia beyond the expected for embryonic epithelium.     

Identifying Lynch syndrome in patients with endometrial carcinoma: shortcomings of morphologic and clinical schemas

It has been suggested that reflex testing for Lynch syndrome (LS) using mismatch repair immunohistochemistry and/or microsatellite instability analysis in newly diagnosed colorectal carcinoma (CRC) patients is an emerging standard of care in the United States. The risk of gynecologic malignancy in women with LS approaches and even exceeds that of CRC. Furthermore, gynecologic malignancies are often the sentinel cancers in these patients. There is significant variation in practice, but some groups have similarly recommended deployment of reflex testing strategies in patients presenting with endometrial cancer (EC). The College of American Pathologists has stated that pathologists should recognize the histologic and clinical features that should prompt at least a recommendation for mismatch repair testing. Morphologic and clinical schemas in EC to identify microsatellite unstable/LS tumors are less refined than the colon-centric schemas (Amsterdam, Bethesda, and MsPath). Studies of LS EC are few and interpretation is limited by recruitment strategies and the myriad of definitions and study designs used. Although serous cell type is used to triage ovarian cancer patients for BRCA screening, cell type correlation in LS is less certain but seems to involve a spectrum of cell types. We review the morphologic and clinical features/schemas in LS EC and highlight limitations of restrictive aged-based screening strategies, uncertainty in current clinical schemas and equivocal results of morphologic studies of LS EC. With uncertainty of histologic and clinical schemas, and following developments in CRC, reflex testing of all/vast majority of newly diagnosed EC for LS should be considered.     

The challenge of apocrine proliferations of the breast: A morphologic approach

Apocrine phenotype in breast is common and can be seen in a broad spectrum of lesions ranging from simple cyst to infiltrating carcinoma. The majority of apocrine lesions of the breast are benign in nature and do not represent a diagnostic challenge; however, there are a few that can cause diagnostic problems, such as the case of apocrine proliferations with atypia and low-grade apocrine ductal carcinoma in situ. Furthermore, the role of atypical apocrine proliferations in the pathway to infiltrating carcinoma is still uncertain, and studies with long-term clinical follow-up are necessary to clarify and understand the significance of these apocrine lesions of the breast. The purpose of this article is to review the most recent literature concerning apocrine lesions, with emphasis on borderline apocrine proliferations.     

Paratesticular cysts with benign epithelial proliferations of wolffian origin

Paratesticular cysts with benign epithelial proliferations (BEPs) are rare. Only 10 cases were found in a series of 431 paratesticular cysts and were classified as follows: cystadenoma, 5; papilloma, 2; and hamartoma, 3. Four cystadenomas showed multiple papillae lined by CD10+ epithelial cells with hyperchromatic nuclei. The remaining lesion showed areas with a microcystic, glandular, cribriform pattern, with small, benign glands without atypia. Urothelial papilloma presented BEPs with cytokeratin (CK) 7+ and CD10+ and CK20- umbrella-like cells. The mural papilloma was lined by proliferative cylindrical cells exhibiting strong CK7 and CD10 expression. The 3 Wolffian hamartomas were characterized by strongly CD10+ epithelium surrounded by smooth muscle cells. The consistent CD10 expression in BEPs of paratesticular cysts suggests a Wolffian origin. The differential diagnosis of paratesticular cysts with BEP vs metastatic prostatic and primary borderline or malignant tumors is discussed.     

Hepatitis C-associated granulomas after liver transplantation: morphologic spectrum and clinical implications

Liver granulomas have been described in biopsy specimens from people with de novo chronic hepatitis C virus (HCV) infection and in allograft biopsy specimens from recipients of transplants for HCV-related liver disease. The latter have not been well studied, and there are no data regarding the prevalence, morphologic spectrum, and clinical significance of HCV-associated granulomas after liver transplantation. We observed granulomas in allograft liver biopsy specimens of 4 (8%) of 53 recipients of transplants for HCV-related end-stage liver disease during a 3-year period. Initial appearance of granulomas ranged from 4 to 41 weeks after transplantation. Lobular and portal nonnecrotizing, epithelioid granulomas and lobular microgranulomas were observed, with the latter predominating. Serum transaminase and alkaline phosphatase levels were significantly higher in patients with granulomas than in age- and sex-matched control subjects, but histologic disease activity, cellular rejection scores, HCV genotypes, viral titers, and retransplantation rate owing to recurrent disease were not significantly different. Our study suggests that a granulomatous response to HCV infection occurs in a subset of patients after transplantation; however, this histologic finding does not portend a worse clinical outcome.     

下颌后静脉的形态学特点及其临床意义

目的:明确下颌后静脉的解剖学特点及其与下颌角之间的解剖关系,为下颌角截骨术中避免损伤下颌后静脉提供解剖学依据。方法:22侧福尔马林固定成人尸体头颈部标本,解剖观测下颌后静脉的走行、构成、长度、外径及其与周围结构的关系。结果:下颌后静脉位于下颌骨后缘,由颞浅静脉和上颌静脉合成。起始处外径为(5.6±2.6)mm,长度为(4.46±2.08)cm。围绕下颌支后缘静脉长度为(4.27±0.80)cm,围绕下颌体下缘静脉长度为(2.02±0.42)cm。结论:在改脸形手术中预切除下颌角的后缘和下缘均有静脉围绕,尤其在下颌支后缘的中段,静脉口径粗大,与下颌支之间仅隔以菲薄骨膜,在下颌角截骨术中要注意避免损伤此静脉。     

Schnitzler's syndrome: a broader clinical spectrum.

Schnitzler's syndrome is characterized by chronic urticaria, recurrent fever, bone pain, and lymphadenopathy in conjunction with a serum IgM M component in a concentration that is usually less than 10,000 mg/L. Complement activation and cryoprecipitation do not appear to be involved. We report two additional patients who share many of the characteristics of this entity. These patients differ from patients previously reported because of the markedly elevated IgM M-component concentration in one patient and the severity of anemia in the second patient. An increased frequency of IgG autoantibodies to interleukin-1-alpha has been reported by other investigators; it has been suggested that an antibody-mediated prolongation of the half-life of interleukin-1-alpha might account for some of the symptoms and signs of this disorder. However, neither the mediators involved in the induction of nonpruritic urticaria nor the role of the IgM M component has been established.     

Mucinous epithelial cysts of the spleen associated with pseudomyxoma peritonei

AIMS: We report two rare cases of neoplastic pseudomyxoma peritonei associated with splenic mucinous epithelial cysts and review previously reported cases of splenic mucinous lesions in order to investigate the extent and implications of such an association. METHODS AND RESULTS: The majority of mucinous lesions of the spleen appear to be associated with pseudomyxoma peritonei. The clinicopathological profile of these cases conforms to that of neoplastic pseudomyxoma peritonei, showing a similar age of onset, outcome and histological features. Most of the cases were associated with a confirmed or suspected appendiceal primary. The immunophenotype (cytokeratin 7 negative; cytokeratin 20 and CEA positive) of the lesions of both our cases, including those in the ovary, was suggestive of a gastrointestinal origin. CONCLUSIONS: Splenomegaly due to cystic intrasplenic mucinous epithelial lesions may occasionally be the presenting feature of pseudomyxoma peritonei or herald tumour recurrence. Mucinous epithelial cysts of the spleen may also precede the development of pseudomyxoma peritonei. All cases of pseudomyxoma peritonei should be investigated for splenic involvement and, conversely, a primary mucinous neoplasm sought elsewhere in the abdomen in all cases of splenic mucinous cysts.     

Hodgkin's and non-Hodgkin's lymphoma: spectrum of morphologic and immunophenotypic overlap.

Immunoperoxidase markers are useful and often essential in distinguishing among lymphocyte-predominant Hodgkin's lymphoma, T-cell-rich B-cell lymphoma, and lymphocyte-rich classic Hodgkin's lymphoma. However, it is becoming increasingly clear that these "entities" are closely related clonal B-lineage neoplasms that may intertransform and/or coexist. We hypothesized that, just as there are cases with morphologic overlap, there would also be immunophenotypic overlap that would be found when a series of such cases is studied in detail. Eight cases of lymphocyte predominant Hodgkin's lymphoma, eight cases of lymphocyte-rich classic Hodgkin's lymphoma, seven cases of T-cell-rich B-cell lymphoma, and four cases of large B-cell lymphoma with focal features of T-cell-rich B-cell lymphoma were examined by the immunoperoxidase technique for expression of CD3, CD15, CD30, CD20, CD57, epithelial membrane antigen, and Epstein-Barr virus latent membrane protein (EBV LMP). All eight of the lymphocyte-predominant Hodgkin's lymphoma cases had CD20+ lymphocytic and histiocytic cells and CD57+ rosettes; however, in two cases, occasional lymphocytic and histiocytic cells were also weakly positive for CD15, CD30, and EBV LMP. Among the eight lymphocyte-rich classic Hodgkin's lymphoma cases, CD15+ Reed-Sternberg (R-S) cells were found in seven; however, in three of these cases rare rosettes of CD57+ cells surrounded the R-S or lacunar cells. In one case of large B-cell lymphoma the malignant cells resembled R-S cells and were CD20+, EBV LMP+, CD30+, CD15-, and surrounded by rosettes of CD57+ T cells. The majority of the cases exhibited the "expected" immunophenotypic patterns; however, the exceptional cases that were found serve to confirm the interrelationship among these clonal B-lineage neoplasms.     

椎板下棘的形态学特点及其临床意义

目的：观测椎板下棘的形态特点，探讨其在显微内窥镜腰椎间盘切除术(MED)中的临床意义。方法：对42具成人干燥椎骨和10件脊柱腰骶段标本的椎板下棘进行观测，分析其对椎间管构成的影响；结合MED术式的通道和操作过程，分析其可能导致的并发症。结果：椎板下棘位于椎板下缘前外近椎弓根下切迹处。可出现于所有胸腰椎，出现率为83．3％；出现率最高在第12胸椎，为52．4％。椎板下棘分布不规则，出现在胸腰段较多。结论：椎板下棘是胸腰椎的骨性结构，有必要给予命名；椎板下棘参与构成神经根管的骨性后壁，可造成神经根受压；后路MED可引起椎板下棘断脱，造成神经根受压或损伤。     

Vimentin在子宫内膜腺癌组织中的表达及其临床意义

目的探讨Vimentin在人子宫内膜腺癌中的表达及其临床意义。方法采用免疫组化方法及Western-blot方法研究子宫内膜腺癌组织及正常子宫内膜组织中Vimentin的表达。结果 Vimentin蛋白表达水平在子宫内膜癌组织中显著高于正常子宫内膜组织(P0.05)。Vimentin在子宫内膜癌组织的表达与年龄,FIGO分期,癌症分化程度,病灶大小以及肌层浸润深度密切相关(P0.05),并与雌激素受体(Estrogen Receptor,ER),孕激素受体(Progesterone Receptor,PR)呈负相关。结论 Vimentin在子宫内膜癌中过表达,提示其可能参与子宫内膜癌的发病机制。     

Refractory anemia with ring sideroblasts associated with marked thrombocytosis: a mixed group exhibiting a spectrum of morphologic findings

Refractory anemia with ring sideroblasts associated with marked thrombocytosis (RARS-T) is a provisional entity in the current World Health Organization classification and is thought to be a myelodysplastic/myeloproliferative neoplasm (MDS/MPN). We analyzed 18 cases of RARS-T. All patients had thrombocytosis (platelet count, 515-1,100 × 10(3)/μL [515-1,100 × 10(9)/L]) and anemia (hemoglobin level, 7.2-12.6 g/dL [72-126 g/L]). Three patients had mild leukocytosis (WBC count, 3,900-16,300/μL [3.9-16.3 × 10(9)/L]). Ring sideroblasts were 8% to 75% in the bone marrow. Megakaryocytes showed a spectrum of morphologic findings. JAK2(V617F) was identified in 9 of 15 cases, including 7 of 9 with thrombocytosis (platelet count, >600 × 10(3)/μL [600 × 10(9)/L]) and 1 with 8% ring sideroblasts. The MPL(W515L) mutation was not detected (n = 9). We conclude that RARS-T is a pathogenetically heterogeneous group of limited diagnostic usefulness. Approximately 60% of cases carry JAK2(V617F)and seem to be closer to an MPN in which ring sideroblasts may be a secondary phenomenon. The remaining cases usually lack the JAK2(V617F)mutation, have a platelet count less than 600 × 10(3)/μL (600 × 10(9)/L), and may represent an MDS or MPN with thrombocytosis of unknown mechanisms.     

The clinical spectrum of narcolepsy with cataplexy: a reappraisal

In the absence of a golden standard for the diagnosis of narcolepsy, the clinical spectrum of disorder remains controversial. The aims of this study were (1) to determine frequency and characteristics of sleep-wake symptoms in patients with narcolepsy with cataplexy, (2) to compare clinical characteristics with results of ancillary tests, and (3) to identify factors that discriminate narcolepsy from other conditions with excessive daytime sleepiness (EDS). We prospectively studied 57 narcoleptics with cataplexy, 56 patients with non-narcoleptic hypersomnia (H), and 40 normal controls (No). Based on suggested and published criteria, we differentiated between narcoleptics with definite cataplexy (N) and narcoleptics without definite cataplexy (possible cataplexy, NpC). Assessment consisted of questionnaires [all patients and controls, including the Ullanlinna Narcolepsy Score (UNS)], polysomnography (all patients), multiple sleep latency test (MSLT) and human leukocyte antigen typing (in most narcoleptics). A new narcolepsy score based on five questions was developed. Data were compared with those of 12 hypocretin-deficient narcoleptics (N-hd). There were significant differences between N and NpC (including mean sleep latency on MSLT), but none between N and N-hd. A score of sleep propensity during active situations (SPAS) and the frequency of sleep paralysis/hallucinations at sleep onset, dreams of flying, and history of sleep shouting discriminated N from H and No (P < 0.001). Cataplexy-like symptoms in H (18%) and No (8%) could be discriminated from 'true' cataplexy in N on the basis of topography of motor effects, triggering emotions and triggering situations (P < 0.001). Our narcolepsy score had a similar sensitivity (96% versus 98%) but a higher specificity (98% versus 56%) than the UNS. Analysis of co-occurring symptoms in narcolepsy revealed two symptom complexes: EDS, cataplexy, automatic behaviors; and sleep paralysis, hallucinations, parasomnias. Low/undetectable cerebrospinal fluid hypocretin-1 levels and a history of definite cataplexy identify similar subgroups of narcoleptics. Specific questions on severity of EDS (SPAS score) and characteristics of cataplexy allow the recognition of subgroups of narcoleptics and their differentiation from non-narcoleptic EDS patients, including those reporting cataplexy-like episodes. The existence of co-occurring symptoms supports the hypothesis of a distinct pathophysiology of single narcoleptic symptoms.     

Post-transplant lymphoproliferative disorders: a morphologic, phenotypic and genotypic spectrum of disease

The post-transplant lymphoproliferative disorders represent a spectrum of life-threatening, generally Epstein-Barr virus-associated lymphoid proliferations which occur in the setting of exogenous immunosuppression following organ transplantation. Histopathological, phenotypic, genotypic and Epstein-Barr virus studies have revealed a broad range of abnormalities. At one end of the spectrum, they are polymorphic polyclonal proliferations with many features of a florid viral infection and, at the other end, they are monomorphic and monoclonal which would fulfill the criteria for a conventional non-Hodgkin's lymphoma, usually of B-cell type. Some patients have both polyclonal and monoclonal lesions and some have two or more monoclonal populations. Finally, because of the varied appearances, different classification schemes have been developed with reported prognostic implications.