Primary cutaneous leiomyosarcoma: a clinicopathological and immunohistochemical study of 7 cases

Primary cutaneous leiomyosarcomas are rare tumors, few series being reported in the current literature. A retrospective study of 7 cases was undertaken to understand the clinicopathological characteristics of these neoplasms and some of their molecular mutations. Histologically, a well-differentiated proliferation of cells of smooth muscle derivation was evident in all cases. The number of mitoses was considered the most important criterion of malignancy (more than 2 for 10 HPF). Smooth muscle actin, desmin, and vimentin were positive in all cases. Immunohistochemical analysis also revealed a positivity for p53 in 3 cases and no reaction for retinoblastoma protein. Research for Epstein-Barr virus was negative in all cases. Three patients developed local recurrences owing to incomplete surgical excision. Recurrent tumors were more atypical and located deeper. No distant metastases were observed. Our results emphasize that cutaneous leiomyosarcomas have an indolent biological course if treated by surgical excision with wide margins. Molecular abnormalities involving tumor suppressor genes are probably involved.     

原发性皮肤恶性淋巴瘤的临床病理和免疫组化研究

研究原发性皮肤恶性淋巴瘤的类型及临床病理特点,方法对４２例ＰＣＭＬ作了形态学观察,免疫表型标记和组织学分型。结果原发性皮肤Ｔ细胞性淋巴瘤（ＰＣＴＣＬ）占６９．０％,其中蕈样霉菌病占７．２％（ＭＦ）、血管中心性淋巴瘤占９．５％皮下指明脂膜炎样Ｔ细胞巴瘤占１９．０％,原发性皮肤Ｂ细胞性淋巴瘤（ＰＣＢＣＬ）占３１．０％;ＰＣＭＬ男女之比为２：１,中位年龄３７岁,ＰＣＴＣＬ的发病年龄小于ＰＣＢＣＬ（     

Primary cutaneous leiomyosarcoma: clinicopathological analysis of 36 cases

Massi D, Franchi A, Alos L, Cook M, Di Palma S, Enguita A B, Ferrara G, Kazakov D V, Mentzel T, Michal M, Panelos J, Rodriguez‐Peralto J L, Santucci M, Tragni G, Zioga A & Tos A P D
(2010) Histopathology 56, 251–262 Primary cutaneous leiomyosarcoma: clinicopathological analysis of 36 cases Aims: Cutaneous leiomyosarcomas (LMS) are rare in comparison with their deep‐seated soft tissue and uterine counterparts, and have been poorly characterized. The aim was to verify whether the clinical behaviour of purely dermal LMS is different from that of LMS with minimal subcutis invasion. Methods and results: Twenty‐one purely dermal LMS and 15 dermal LMS with minimal subcutis extension were analysed. Tumours developed in 27 men and nine women (age range 29–91 years); most tumours showed a fasciculated (n = 23), pilar‐type (n = 12) and pleomorphic (n = 1) pattern. During the follow‐up period (range 2–192, mean 41 months) recurrences occurred in 1/16 (6.2%) of tumours confined to the dermis and in 2/11 (18.1%) tumours with minimal subcutis extension. The three recurrent tumours were high‐grade LMS, two of which exhibited myxoid areas. One patient with a pleomorphic dermal LMS with minimal extension into fat developed distant metastases 15 years after diagnosis. Conclusions:  For LMS involving the skin, it is advisable to recognize and indicate in the histopathology report the depth of dermal and/or subcutaneous extension, since even minimal subcutaneous involvement may be associated with late local recurrences and/or distant metastases, and therefore appropriate and long‐term follow‐up is needed.     

Primary lymphoma of the thyroid: a clinical, histological and immunohistochemical study of 20 cases

Twenty cases of malignant lymphoma arising in the thyroid gland were studied clinically, histologically and immunohistochemically. Nineteen cases were non-Hodgkin's lymphoma (15 diffuse and four follicular lymphoma) and one was a plasmacytoma. Immunohistochemical analysis of the lymphomas using paraffin-embedded sections disclosed that 17 lymphomas were B-cell type and two were T-cell type. The plasmacytoma was of IgG kappa type. The large majority of the lymphomas were associated with an underlying chronic thyroiditis. The 5-year survival rate of the patients was 70%. An unfavourable diagnosis was more likely when the tumour was diffuse rather than follicular, when it was of diffuse large cell type or of immunoblastic type and when there was cervical lymph node involvement.     

Chondroid syringoma: a histological and immunohistochemical study of 15 cases

Fifteen cases of chondroid syringoma have been studied histologically and by immunohistochemical methods in an attempt to establish their phenotypic profile and to elucidate their histogenesis. The epithelial elements were classified as tubuloglandular, solid nests and stromal cells. The inner cell layers of tubuloglandular components had distinct epithelial features, expressing cytokeratin, carcino-embryonic antigen and epithelial membrane antigen. The outer cell layers expressed vimentin, S-100 protein, neuron-specific enolase and muscle-specific actin and were negative for epithelial markers. The immunophenotypes of stromal cells and solid nests were similar to those of the outer cell layers. These data suggest that the stromal components may derive from the outer cell of tubuloglandular elements and that myo-epithelial cells have an important role in the histogenesis of these lesions and in their mesenchymal matrix production.     

Primary ovarian leiomyosarcoma. An immunohistochemical and ultrastructural study

Primary sarcoma of the ovary is rare, and only 16 reported examples of leiomyosarcoma are known to us. We encountered a primary ovarian leiomyosarcoma in a 58-year-old woman. The tumor was characterized by interlacing bundles of plump spindle cells that showed immunoreactivity for desmin, vimentin, and muscle-specific actin. Ultrastructural features included abundant smooth-muscle-type filaments, notched nuclei, pinocytotic vesicles, and extracellular collagen. This case illustrates the utility of ancillary techniques in the classification of pure ovarian sarcomas and leads us to propose ultrastructural and immunohistochemical criteria for primary ovarian leiomyosarcoma.     

Primary leiomyosarcoma of bone. An immunohistochemical and ultrastructural study

Primary leiomyosarcoma of bone is extremely rare. A 60-year-old woman had a mass in the right femur that was studied immunohistochemically and by electron microscopy. Human smooth-muscle actomyosin was detected in tumor cells, but human skeletal-muscle myoglobin and lysozyme (muramidase) were not. Electron microscopy of the tumor showed findings suggestive of a smooth-muscle origin, such as myofilaments, dense bodies, pinocytotic vesicles, and basement membrane. The results were diagnostic of leiomyosarcoma rather than rhabdomyosarcoma, fibrosarcoma, or malignant fibrous histiocytoma, which are similar neoplasms. We believe that ours is the first case of primary leiomyosarcoma of the bone proved by immunohistochemistry.     

Primary leiomyosarcoma of bone--a clinicopathologic study of 8 uncommon cases with immunohistochemical analysis and clinical outcomes

Primary leiomyosarcoma of bone is a rare and a diagnostically challenging tumor entity. Over a 7-year period, we identified 8 such cases that fulfilled the diagnostic criteria in 6 men and 2 women, with age ranging from 25 to 59 years (mean, 42.7 years). All cases were noted in the lower limbs, including femur and tibia as the commonly involved bones in 4 and 3 cases, respectively. On radiography, the most consistent feature was a solitary osteolytic lesion with cortical destruction, unassociated with matrix formation. On histopathology, all cases showed spindly sarcomatous cells, mostly arranged in fascicles and whorls. Of 8 cases, 6 (75%) were of high grade. Prominent vasculature was noted in 5 cases. Two cases displayed focal mineralization, including calcification and heterotropic woven bone formation in 1 case each, but lacked malignant osteoid or chondroid matrix. One case showed osteoclast-like giant cells. On immunohistochemistry, smooth muscle actin was diffusely positive in all cases (100%), desmin was positive in 6 (75%) of 8 cases, and h-caldesmon was positive in 5 (83.3%) of 6 cases. Five cases underwent surgery, including 3 amputations and 2 wide excisions. One case underwent chemotherapy. On follow-up, 5 cases developed metastasis, including 1 case with another, who died within 17 and 5 months. Leiomyosarcoma of bone is uncommon and diagnostically challenging. An index of suspicion is necessary for this diagnosis, especially in cases of lytic, destructive bone lesions, unassociated with matrix production, that show spindly sarcomatous cells on histopathology. Immunohistochemical analysis, including an optimum panel formed by smooth muscle actin (diffuse positivity), desmin, and h-caldesmon, is necessary for substantiating this diagnosis. Surgery forms the treatment mainstay. The prognosis appears to be dismal.     

Sweet''s syndrome: histological and immunohistochemical study of 15 cases.

Conventional histology and immunoperoxidase staining for fibrin, immunoglobulins, and complement components were used to look for evidence of cutaneous vasculitis and immune complex deposition in Sweet's syndrome. These features were not identified in any of the 15 cases studied. The lack of any vasculitis emphasises the distinctive character of Sweet's syndrome when compared with certain spontaneous and experimentally induced inflammatory skin lesions, and may imply a similarly distinctive pathogenesis.     

Primary spinal paragangliomas: a clinicopathological and immunohistochemical study of six cases

Spinal paragangliomas are uncommon neoplasms and subject of much debate regarding the factors governing their biological behaviour. We describe the clinicopathological and immunohistochemical (IHC) features of six cases of spinal paraganglioma. The mean age of patients was 40 years (range 20-60 years) with a male to female ratio of (2:1). Majority presented with low backache, sphincter disturbances and sensory symptoms. All tumors were intradural in the cauda region one of them extending to the filum. Only one showed focal extradural extension on microscopy. Gross total resection of tumors was possible in all cases. Histologically four showed classical 'zell-ballen' pattern and two revealed an ependymal morphology. On immunohistochemistry, in all the six cases the chief cells were intensely labeled by antibody to chromogranin but not for GFAP while staining for synaptophysin was less intense and variable in five. Sustentacular cells in all cases showed strong expression for S-100 protein and chief cells were stained light in three cases. Low MIB-1 labeling index of 0.01-2% was noted in five cases and in the sixth it was 5%. None of the tumours recurred. Immunohistochemistry assisted in differentiating these relatively benign neural crest tumours from the more aggressive spinal ependymomas.     

Primary spinal paragangliomas: a clinicopathological and immunohistochemical study of 30 cases

Aims: Extra-adrenal paragliomas are neoplasms which have been the subject of much debate regarding parameters to establish their biological behaviour. This study describes the clinicopathological and immunohistochemical features of 30 cases of spinal paragliomas. Methods and results: There were 15 male and 15 female patients. The median age at diagnosis was 46 years (range 20–74 years). Fourteen patients presented with back pain, two with numbness of the lower extremities, one with difficulty in walking and one with spinal cord compression. Nineteen tumours were located in the lumbar region, six in the cauda equina, two in the filum terminale, two in the thoracic region and one in the cervical region. All patients underwent gross total excision. The size of the tumours ranged from 10 to 50 mm. Histologically, 18 neoplasms showed alveolar (Zellballen) pattern, seven a spindle component, two eosinophilic granular cells suggestive of oncocytic metaplasia, two melanin pigment and one ganglion cells. Positive immunohistochemical results include: neuron-specific enolase 23/23 (100%), synaptophysin 21/23 (91%), S100 protein 22/23 (95%, sustentacular cells), leu-enkephalin 11/23 (47%), somatostatin 8/23 (34%), focal glial fibrillary acidic protein 7/23 (30%), focal keratin 5/23 (21%), neurofilament proteins 3/23 (13%) and adrenocorticotrophic hormine (ACTH) 1/23 (4%). Follow-up information obtained in 20 patients show 17 patients alive over a period of 6–216 months. One patient had bone metastases. Two patients died of unrelated causes, including one of congestive heart failure and one of myocardial infarction. Conclusions: In our experience, spinal paragangliomas behave as slow-growing tumours susceptible to potential cure by total excision. We agree with the current World Health Organization (WHO) classification as grade I tumours. Less than 1% may be locally aggressive. Spinal paragangliomas immunoreact not only for conventional neuroendocrine markers but also for peptides including somatostatin and ACTH and focally for the epithelial marker keratin.     

Dedifferentiated leiomyosarcoma of the intestinal tract: histological,ultrastructural and immunohistochemical examinations

Summary Six cases of dedifferentiated leiomyosarcoma of the small and large bowel are presented with histological, ultrastructural and immunohistochemical examination. One case arose in the jejunum, two in the ileum, and the other three in the large intestine. The tumours were submucosal in four cases with large areas of ulceration; two were polypoid. Four tumours showed typical leiomyosarcomatous appearance with dedifferentiated components and two were typical leiomyosarcomas at the primary site with differentiated components only in metastatic foci. By immunohistochemistry, typical leiomyosarcomatous areas showed a positive reaction for muscle-specific actin (MSA), MB1, MB2 and myosin. In contrast, desmin-positive cells were scattered throughout the tumour or were not present. Tumour cells in dedifferentiated components were positive for alpha-1-antitrypsin and alpha-1-antichymotrypsin in all cases but one; neuron specific enolase, MB1, MB2 and myosin were positive with variety. MSA was faintly positive in only a few tumour cells of two cases and desmin was not detected in any of the cases studied. Ultrastructurally, tumour cells in typical leiomyosarcomatous areas demonstrated evident smooth muscle features, although in dedifferentiated areas they lacked such features except in one case. Our results indicate that dedifferentiated elements may derive from ordinary leiomyosarcoma and loose muscle features due to dedifferentiation.     

Primary gastric lymphoma—a tumour of mucosa-associated lymphoid tissue. A histological and immunohistochemical study of 36 cases

A series of 36 cases of non-Hodgkin's lymphoma of the stomach have been analysed using routine histological techniques and immunohistochemistry. All cases were categorized as follicle centre cell lymphomas. Apart from two cases who had nodal lymphomas followed by gastric lymphomas, all cases appeared to represent primary lymphoma of mucosa-associated lymphoid tissue. It is proposed that the morphology and behaviour of these tumours reflect their origin from gut-associated lymphoid tissue. Physiologically well-differentiated examples show monotypic plasmacytic differentiation. Infiltration of gastric glands by follicle centre cells forming characteristic lympho-epithelial lesions is, we believe, a pathognomonic feature of primary gastric lymphoma. The spread of these tumours is within the mucosa-associated lymphoid tissues involving, in particular, the nasopharynx and lung but seldom spreading to peripheral lymph nodes or bone marrow. This concept of gastric lymphomas as primary neoplasms of gut-associated lymphoid tissue has important implications with respect to the investigation and treatment of this disease.     

Non-epithelial cellular components in eccrine spiradenoma: a histological and immunohistochemical study of 20 cases

In this study, 20 cases of eccrine spiradenoma have been examined using monoclonal antibodies to identify the nature of the epithelial, as well as the non-epithelial, cellular components of this tumour. The results indicate that there is striking similarity between the epithelial cells of eccrine spiradenoma and the normal cells lining eccrine apparatus. An interesting finding was the presence of abundant T-lymphocytes and Langerhans' cells within the tumour lobules. Additionally, endothelial-lined vascular channels and neurofibrils were prominent in larger lesions.     

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。     

Primary mucosal malignant melanoma: an immunohistochemical study of 12 cases with comparison to cutaneous and metastatic melanomas

Immunohistochemical analysis of 40 formalin-fixed, paraffin-embedded malignant melanomas (12 primary mucosal, 16 primary cutaneous, and 12 metastatic cutaneous) was performed to study the possible differences in immunostaining profiles according to location. The majority of melanomas were reactive with a polyclonal antibody to S100 protein (P-S100; 85%), a monoclonal melanoma-specific antibody (HMB-45; 88%), and a monoclonal antibody to vimentin (90%), and there were no differences in staining profiles for these antibodies by anatomic location. In contrast, while 13 of 16 cutaneous melanomas (81%) and ten of 12 metastatic melanomas (83%) were reactive with a monoclonal antibody to S100 protein (MoAb-079), only five of 12 mucosal tumors (42%) showed positive staining for MoAb-079. Similarly, 14 cutaneous melanomas (88%) and 11 metastatic melanomas (92%) showed positive staining for neuron specific enolase (NSE), while only four mucosal melanomas (33%) were NSE-positive. Of the 40 melanomas, all but two were reactive with either P-S100, MoAb-079, or HMB-45. These findings suggest that MoAb-079 and NSE may be less sensitive markers than P-S100 and HMB-45 for routinely processed mucosal melanomas as compared with cutaneous and metastatic tumors.