Sclerosing stromal tumour of the ovary--a clinicopathologic spectrum

Majority of the Sclerosing stromal tumours of the ovary documented in the literature are single case reports. We report a series of 4 cases. Among the 4 cases encountered the mean age at presentation was 22.2 years. The clinical presentation varied from asymptomatic mass per abdomen (2 cases), menorrhagia (1 case) and amenorrhoea (1 case). The tumour was unilateral in all the cases with an average size of 10 cms. Grossly the appearances varied from a solid, partly cystic, edematous tumour (2 cases) to solid,firm tumour with yellow flecks (1 case) to unilocular cystic tumour (1 case). Microscopically, the tumour was characterized by cellular pseudolobules composed of a disorderly admixture of collagen-producing fibroblasts and lipid rich lutein cells with shrunken nuclei. In one case the lutein cells had a robust appearance with abundant cytoplasm and vesicular nuclei. The pseudolobules were very vascular and separated by hypocellular dense to oedematous fibrous tissue. Frozen section demonstrated fat in luteinized cells in 3 cases.     

Sclerosing stromal tumour of the ovary--a case report.

Sclerosing stromal tumour (SST) of the ovary is a rare, benign tumour of the ovary, distinct from thecoma-fibroma group of tumours because of predominant occurrence below 30 years of age, lack of hormonal manifestations and histologic heterogenity. A case of 17-year-old female patient is described in the present article. The differential diagnosis is also discussed.     

Sclerosing perineurioma: a clinicopathological study of five cases and diagnostic utility of immunohistochemical staining for GLUT1

We reviewed five cases of sclerosing perineurial tumor of the hand. Four patients were male and one was female with ages ranging from 11 years to 49 years (mean 26 years). The predominant reason for consultation at the outpatient clinic was a slowly growing painless mass. The sites of involvement were the thumb in two cases, and the ring finger, middle finger and palm in one case each. The lesions were hard and firm, well-circumscribed white masses with a fibrous consistency ranging from 1.2 cm to 4.0 cm (mean 2.5 cm) in maximum dimension. Microscopically, all the tumors were composed of thick collagen and variable numbers of small, epithelioid cells exhibiting corded, trabecular and whorled growth patterns. Electron microscopy showed long cytoplasmic processes with a discontinuous basal lamina and occasional pinocytotic vesicles in the tumor cells. Immunohistochemically, most of the tumor cells were positive for epithelial membrane antigen, vimentin, collagen type IV and CD10, but not for S-100 protein, CD34, desmin and cytokeratin. We also observed that the tumor cells were positive for the human erythrocyte glucose transporter (GLUT1) antigen, suggesting that GLUT1 may be a useful marker for the identification of sclerosing perineurioma.     

Necrotizing lymphadenitis: A clinicopathological and immunohistochemical study of four familial cases and five recurrent cases

Summary We report the clinicopathological and immunohistological findings of nine cases of necrotizing lymphadenitis, consisting of four cases of familial infection and five cases of recurrence. Fever, cervical lymphadenopathy, leucopenia and swelling of the tonsils are characteristic clinical findings. Morphological features of the lymph nodes include the presence of immunoblasts, plasmacytoid T cells, histiocytes and macrophages, the latter with phagocytized nuclear debris derived from degenerated lymphocytes. However, granulocytes are generally absent. Ultrastructurally, tubuloreticular structures are observed not only in lymphoid cells, but in vascular endothelial cells. Immunological studies of peripheral blood using monoclonal antibodies disclose that CD 8⁺ (Leu 2a⁺: suppressor/cytotoxic) cells predominate at the onset, but they gradually decrease with the clinical course and the ratio of CD 4⁺CD 8⁺ (helper:suppressor) increases as the disease progresses. However, in the affected lymph nodes, CD 4⁺ (Leu 2a⁺:helper/inducer) cells often increase with the clinical progression, but the ratio of CD 4⁺CD 8⁺ in the lymph nodes does not correlate with clinical progression. In addition, Ki-67⁺CD 8⁺ cells are more often seen than Ki-67⁺CD 4⁺ cells. It is suggested that necrotizing lymphadenitis is an infectious disease in which CD 4⁺ cells are disrupted and CD 8⁺ cells undergo transformation to blastoid cells. This results in a change in the ratio of T subsets.     

An immunohistochemical and clinicopathological study of gastrointestinal stromal tumors

Immunohistochemical and clinicopathological features of 58 gastrointestinal stromal tumors (GIST) were studied. One occurred in the esophagus, 41 in the stomach, nine in the small intestine, and seven in the large intestine. By using indirect immunoperoxidase staining for Cajal cell markers (c-kit protein and CD34), smooth muscle markers (alpha-smooth muscle actin, desmin, heavy caldesmon and calponin) and Schwann cell markers (S-100 protein and Leu 7), GIST were classified into five groups, such as Cajal cell type (n = 9), myogenic type (n = 5), Schwann cell type (n = 2), mixed cell type (n = 40) and undifferentiated type (n = 2). c-kit Protein (42/58; 72%) and CD34 (45/58; 78%) were commonly and diffusely expressed in GIST. Novel smooth muscle markers, caldesmon (29/58; 50%) and calponin (18/58; 31%), were useful in detecting myogenic characters of GIST. S-100 protein was expressed in 16 (28%) tumors, two of which were also reactive with Leu 7 (CD57). Three small bowel tumors with skeinoid fibers expressed the Cajal cell markers, and were categorizable in GIST. Clinicopathological analyses using aggressive (n = 21) and non-aggressive (n = 21) GIST indicated that the malignant potential was correlated with the intestinal location, large tumor size, high cellularity, necrosis, solid (non-interlacing bundled) pattern of growth, negativity of c-kit protein and/or CD34, high mitotic count, and high MIB-1 labeling.     

Micronodular thymic carcinoma with lymphoid hyperplasia: a clinicopathological and immunohistochemical study of five cases

Five cases of an unusual variant of thymic carcinoma are described, which represent the counterpart of the so-called micronodular thymoma with lymphoid hyperplasia. The patients were three men and two women aged 42-78 years (mean 64 years). Three patients were asymptomatic and the tumors were found incidentally on chest radiographs performed for unrelated reasons. Two patients complained of dyspnea, chest pain and shortness of breath prompting further investigations. The tumors ranged in size from 3.2 to 10.0 cm and were described as infiltrative masses often invading adjacent structures. Prominent cystic changes were not identified. Histologically, the neoplasms were composed of epithelial tumor cells arranged in a micronodular growth pattern set in a stroma showing florid lymphoid hyperplasia. Contrary to micronodular thymoma, the epithelial cell component of the present cases showed unequivocal signs of malignancy characterized by cytological atypia and increased mitotic activity. Immunohistochemical studies showed the lymphoid component to be of mixed B- and T-cell lineage. None of the patients had a history of myasthenia gravis or other autoimmune disorder. Follow-up revealed that 4 patients were alive and well 3-26 months after diagnosis while 1 patient was dead of disease 21 months after diagnosis. The tumors in this series represent a distinct subtype of thymic carcinoma histologically strongly resembling micronodular thymoma with lymphoid hyperplasia. Awareness of this type of thymic carcinoma is important in order not to dismiss this tumor for a neoplasm of lower-grade malignancy.     

34例脊索瘤的临床病理及免疫组织化学研究

为了解脊索瘤的临床病理及免疫组化特点，我们对34例脊索瘤进行了临床病理学及免疫组织化学标记的研究，并以5例软骨肉瘤作为对照。根据有无软骨样区域，将脊索瘤分为两型：软骨样脊索瘤（14例）和经典型脊索瘤（20例）。软骨样脊索瘤发病年龄（平均40．9岁）较经典型（平均51．1岁）年轻。7例（50％）软骨样脊索瘤发生于骶尾部，4例（28．6％）发生于颅底蝶枕部。免疫组化标记染色显示：34例脊索瘤均对细胞角蛋白呈阳性反应，16例（47．1％）对上皮性膜抗原阳性；而5例软骨肉瘤均对细胞角蛋白和上皮性膜抗原阴性。波形蛋白及S-100蛋白在大部分脊索瘤及5例软骨肉瘤中均呈阳性巨应。其结果验证了脊索瘤的双重特性──上皮性及间叶性；同时也说明了免疫组化染色对鉴别脊索瘤和软骨肉瘤很有帮助。     

卵巢硬化性间质瘤临床病理及电镜和免疫组化分析

目的探讨卵巢硬化性间质瘤(sclerosing stromaltumer of ovarg,SST)的临床病理、电镜及免疫组化特点。方法通过11例SST的临床、形态学特征、免疫组化及其中的5例电镜观察和10例随访。结果 11例中30岁以下8例,10例有月经紊乱,1例早熟,5例伴有腹水。光镜显示假小叶结构及小叶间疏松水肿的结缔组织和致密的胶原纤维,瘤细胞形态多样,可见印戒样细胞,血管丰富。电镜见有三类瘤细胞:分别为类似储脂细胞,纤维母细胞及原始未分化间叶细胞,瘤细胞间或周围有大量的毛细血管。组织化学:PAS全部(-);免疫组化:α-inhibin7例(+),4例(-);ER4例(+),7例(-);CR3例(+),8例(-);vimentin、SMA、PR均(+);CK、EMA均(-)。随访:术后月经均恢复正常,已婚未育2例术后都怀孕生子,未婚者中术后有2例结婚并已生子。结论 SST是一种具有特殊组织学结构的少见卵巢良性肿瘤,术后不复发,预后好。病理诊断应注意与卵巢印戒细胞间质瘤、卵泡膜细胞瘤、卵巢纤维瘤、Krukenberg瘤等鉴别。     

Ureteral endometriosis: clinicopathological and immunohistochemical study of 7 cases

Ureteral endometriosis is a rare yet important entity that can lead to renal failure due to silent obstruction of the ureter. Awareness of clinical and morphologic features can help in early detection and treatment. We analyzed the clinical, pathologic, and immunohistochemical findings of 7 cases of ureteral endometriosis. Mean age of patients was 51 years. All patients presented with hydroureter, accompanied in the most cases by hydronephrosis. Superimposed pyelonephritis was experienced by 2 of 7 patients. Most patients (4 of 7) had previously undergone total abdominal hysterectomy with bilateral salpingo-oophorectomy. In 6 of 7 cases, endometriosis involved the left ureter. The distal one third of the ureter was involved in 6 cases, whereas the middle third was involved in 1 case. In 4 cases, endometriosis was located extrinsic to the ureter, whereas in 3 cases, the ureter showed intrinsic involvement by endometriosis. One case showed simple endometrial hyperplasia. Surgical management included nephrectomy in 2 cases, distal ureterectomy with reimplantation in 3 cases, ureteral stent placement followed by ureteroureterostomy in 1 case, and relief of ureteral obstruction by resection of pelvic endometrioma in 1 case. Immunostains for cytokeratin-7 (CK7) and progesterone receptor (PR) were positive in all of the cases, whereas immunostains for estrogen receptor (ER) were positive in 83% of cases and immunostains for CK20 were negative in all cases. CA125 immunostains were positive in 67% of cases. The stromal cells were positive for CD10, ER, and PR immunostaining. Our findings suggest that the diagnosis of ureteral endometriosis is preceded in most cases by hysterectomy and bilateral salpingo-oophorectomy, possibly because of prior symptoms related to adenomyosis or pelvic endometriosis and that ureteral endometriosis has a strong predilection for involvement of the lower third of the left ureter. Ureteral endometriosis should be included in the differential diagnosis of obstructive ureteral lesions in women, particularly those involving the lower third of the left ureter, even in postmenopausal patients. Immunostains for ER, PR, CK7, CA125, and CD10 can be helpful in challenging cases.     

Prognostic indicators for gastrointestinal stromal tumours: a clinicopathological and immunohistochemical study of 108 resected cases of the stomach

AIMS: Whether immunohistochemical markers increase accuracy in predicting prognosis for gastrointestinal stromal tumours (GISTs) remains uncertain. However, past studies have used only small, heterogeneous patient groups. Our aim was to test previously studied and more novel morphological features as well as four immunohistochemical markers as prognostic indicators amongst a large cohort of surgically resected, gastric GISTs. METHODS AND RESULTS: Tissues from 127 gastric mesenchymal tumours were collected retrospectively and subjected to repeat histological assessment and immunophenotyping. Further immunohistochemistry was performed for Ki67, p53, Bcl-2 and cyclin D1. Complete follow-up data were collected for 108 patients with immunophenotyped diagnoses of GIST (i.e. c-kit+ tumours). At the census point, 52 patients were alive, 24 had died from their GISTs and the remainder of other causes. Univariate analysis showed the following predicted for shorter disease-specific survival: size > or =50 mm; necrosis, no intratumoral lymphocytes; mitotic count > or =5/50 high power fields; Ki67 labelling index > or =5%; p53 immunopositivity. Of these variables, multivariate analyses showed only mitotic count and, to a lesser extent, Ki67 labelling to be independent prognostic indicators. CONCLUSIONS: Mitotic count remains the best predictor of outcome following surgical resection of gastric GISTs. Ki67 immunohistochemistry does not provide better prognostication and p53, Bcl-2 and cyclin D1 immunohistochemistry provide no additional prognostication.     

乳头状汗管囊腺瘤5例临床病理学分析

目的 探讨乳头状汗管囊腺瘤(SCAP)的临床病理特征和诊断要点。方法 分析5例SCAP临床资料，观察其组织学形态及免疫表型特征。结果 本组男4例、女1例；发病年龄0～14岁，临床上皮损表现为数个丘疹呈线状排列或孤立性结节、斑块。组织学特征：大小不一的乳头状突起衬以两层上皮细胞。免疫表型：SCAP绝大多数内层柱状细胞CK7、CK19阳性，外层立方细胞CKS/8、CK7阳性。结论 SCAP是一种少见的良性皮肤错构瘤，鉴别诊断包括乳头状汗腺囊腺瘤、管状大汗腺腺瘤。     

横纹肌样型脑膜瘤临床病理学分析

目的探讨横纹肌样型脑膜瘤（rhabdoid meningioma,RM）的临床病理特征。方法对4例RM进行病理形态学观察及免疫组化分析。结果4例发病年龄为11—40岁,平均27岁,3例为单纯型,1例为混合性。瘤细胞呈圆形、多边形,富含胞质呈嗜酸性或嗜中性,均见有胞质或核内假包涵体。瘤细胞呈弥漫性、腺泡状和乳头状排列。免疫组化均表达vimentin和EMA,而HMB45和MG均阴性,仅有1例GFAP呈小灶性阳性。结论RM的诊断应依据细胞学和组织结构的特征,辅以免疫组化分析。     

Spermatocytic seminoma. A clinicopathological and immunohistochemical study of 7 cases

Spermatocytic seminoma is an uncommon tumor, representing less than 1% of the testicular tumors, occurring most often in old patients. We report 7 cases of this entity. The average age at presentation was 66 years. Tumors had a polymorphic appearance with small, intermediate and large cells, and "spireme" figures. They were pure, with no sarcomatous component. In all cases, the tumor was limited to the testis. In the peritumoral tissue, there was no intratubular germ cell proliferation, and no atrophic testis. Immunostaining was negative for all the classical antibodies tested (cytokeratins, PLAP, lymphoid markers), but all the cases expressed c-kit (100%). This membranous positivity was focal in 4 cases, very strong, and diffuse in the 3 others. Spermatocytic seminoma must be recognized, because its favorable evolution in absence of a sarcomatous component. Adequate treatment consists of orchidectomy alone. Positive staining for c-kit may be helpful for the diagnosis of spermatocytic seminoma.     

Isolated thoracic aortitis: clinicopathological and immunohistochemical study of 11 cases

Isolated thoracic aortitis (ITA) is diagnosed in a variable proportion of patients operated on for dilation/aneurysm of ascending aorta. The etiopathogenesis of ITA remains unclear.We studied 11 cases of ITA in order to determine the role of IgG4-mediated immune responses in its pathogenesis.The series included nine women and two men aged 52–79 years. All patients developed aortic incompetence due to dilation/aneurysm of ascending aorta. None of the patients had a history of IgG4-related disease neither did they develop features of such disease during the follow-up period. The microscopic findings included the presence of lymphoplasmacellular fibrosing infiltrate of varied intensity involving the adventitia and media of aorta. This inflammation was associated with severe medial elastic fiber defects. Obliterative phlebitis of the vasa vasorum was absent. Immunohistochemically, the inflammatory infiltrate comprised T- and B-lymphocytes as well as plasma cells. The plasma cell population was polyclonal with a predominance of IgG-producing cells. In all the cases, IgG4-producing plasma cells were detected. In five cases, the count exceeded 20 cells per high-power field. The IgG4/IgG ratio ranged from 0.07 to 0.98 (median 0.55). In six cases with the ratio >0.50, severe adventitial fibrosis was present.To the best of our knowledge, ours is the first study focused on investigating the role of IgG4-positive plasma cells in the development of ITA. Our results suggest that a subset of ITA may represent aortic manifestation of IgG4-related disease. Further research is necessary in order to clarify this issue.     

Endometrial stromal sarcoma: clinicopathological and immunophenotype study of 18 cases

Malignant tumors of the uterine corpus are uncommon. They originate from the endometrial stroma, smooth muscle, blood vessels, or from a mixture of them. The objective of this article was to know the frequency and the clinical, morphologic, and immunophenotype characteristics of the endometrial stromal sarcoma (ESS). We reviewed the cases of ESS observed from 2002 to 2008 at the Pathology Unit of the General Hospital of Mexico. The following data were analyzed: age, clinical stage, degree of differentiation, and immunophenotype. We found 18 cases, and the average age of patients was 48.6 years; 66% were in clinical stages 1 and 2. Fifteen cases (83.3%) were classified as low-grade sarcomas and 3 (16.6%) as high-grade or undifferentiated sarcomas. We determined immunohistochemical markers in 17 cases; receptors to estrogens were positive in 5 (29.4%) and to progesterone in 9 (52.9%). CD10 was expressed in 10 (58.8%) and p53 in 11 cases (64.7%). Two cases were associated to primary tumors of the ovary (papillary cystadenocarcinoma). In conclusion, ESS was present at 0.6% in our institution; and most were low grade. Expression of markers, such as p53, CD10, and hormonal receptors, was positive.     

74例胃肠道间质瘤临床病理与生物学行为评价

目的　探讨在胃肠道间质瘤(GIST)的病理诊断和预后分析上采用一种简单实用且重复性好的病理学“标准”,以利于GIST的日常病理诊断和生物学行为评价及指导治疗,并对Fletcher等推荐的GIST生物学行为评价表进行评估。方法　85例消化道间叶组织肿瘤,复习其病理形态学并应用CD117、CD34、平滑肌肌动蛋白(SMA)、结蛋白、S 100等进行免疫组织化学标记,结合 31例随访资料进行分析。结果　85例消化道间叶组织肿瘤中,GIST74例,平滑肌瘤和交界性平滑肌瘤 8例(食管),平滑肌肉瘤 1例(直肠 ),神经鞘瘤 1例 (胃 ),恶性纤维组织细胞瘤 1例 (肠系膜 )。74例GIST中,发生在胃和小肠的分别为 34例和 30例,占 86. 5%,食管 3例,胃肠道外(肠系膜、网膜、后腹膜)7例。年龄 23~80岁,平均 52 5岁, 40岁以上者占 85%,男性 45例,女性 29例。镜下观察:梭型细胞型 48例,上皮样细胞型 10例,混合细胞型 16例。瘤细胞呈长、短梭形和圆形,胞质丰富弱嗜酸性,排列呈旋涡状、栅栏状或弥漫巢状。免疫组织化学: 85例消化道间叶组织肿瘤波形蛋白均阳性,其中 74例表达CD117,诊断为GIST,表达形式有弥漫胞膜 /胞质强阳性、散在阳性、胞质点状着色等,其中 54例同时表达CD34 (阳性率 72. 9% ), 25例表达SMA, 5例表达结蛋白, 5例表达S 100蛋白。在 85例