新疆kaposi肉瘤43例临床及病理学分析

目的：回顾性研究新疆地区发现的43例卡波西肉瘤临床病理学特点。方法：对43例kaposi肉瘤进行了光镜检查。结果：新疆卡波西肉瘤属于经典卡波西肉瘤，基本损害为斑片、斑块及结节，主要位于四肢末端。组织学以梭形细胞增生、血管瘤样结构、红细胞外溢及含铁血黄素沉积为主。结论：不同临床类型卡波西肉瘤在临床和组织学上均表现为病谱性特点，提示kaposi肉瘤可能为一种病谱性疾病。     

Lobular panniculitis at the site of subcutaneous interferon beta injections for the treatment of multiple sclerosis can histologically mimic pancreatic panniculitis. A study of 12 cases

Background:  Thrombosis, mucinosis and necrosis are well-described complications of subcutaneous interferon beta injections.
Methods:  We report 12 incisional biopsies from subcutaneous interferon beta injection sites in 12 multiple sclerosis (MS) patients from a single neurologist's practice.
Results:  We identified abscesses (two cases) or induration (two cases) in acute clinical lesions and lipoatrophy (eight cases) in chronic lesions (biopsied over a year after symptom onset at injection sites). Biopsies from three acute lesions showed vascular thrombosis, dermal mucinosis, lobular neutrophilic panniculitis, necrosis, calcification and hemosiderin deposition (biopsied 2 weeks to 2 months after symptom onset). Two cases contained sterile abscesses. Five of the eight chronic cases presented as hard, indurated lipoatrophy with livedo reticularis. Their biopsies showed subcutaneous calcification and lipoatrophy. Biopsies from the early calcific suppurative and late calcific atrophic phases histologically resembled the early and late phases of subcutaneous saponification in pancreatic panniculitis.
Conclusions:  Reactions at the site of subcutaneous interferon beta injections are common. Lipoatrophy can be clinically identified in 39 of 85 MS patients (46%) receiving subcutaneous interferon beta injections for 1 year or longer in our practice. A reaction to interferon should be considered in the differential diagnosis of biopsies that show features of pancreatic panniculitis.     

In situ study of chemokine and chemokine-receptor expression in Kaposi sarcoma

Tissue expression of CC and CXC chemokines and chemokine receptors was investigated in 6 cases of classic non-AIDS Kaposi sarcoma (KS) using immunohistochemistry and RNase protection assay (RPA). Immunostaining of frozen sections of KS skin biopsies revealed that KS spindle cells express several chemokine receptors. In KS nodules, almost all KS spindle cells were intensely stained for CXCR4 and CCR5. Other chemokine receptors as CCR1, CXCR3, and CCR2 were also detected in the large majority of KS spindle cells. A minority of KS spindle cells also expressed the fractalkine receptor (FK-R) CX3CR1.The immunohistochemical findings were confirmed at RNA level. In fact, the RNase protection assay (RPA) revealed in 6 of 6 cases the presence of consistent amounts of mRNAs for CXCR4 and CCR1 and in 5 of 6 cases also for CCR5 and CXCR3.Expression of chemokine receptors by KS cells was associated with chemokine production within the lesions. In the same cases, RPA demonstrated the presence of mRNAs for MCP-1, RANTES, IP-10, MIP-1alpha, and MIP-1beta. Chemokine-producing cells, as detected by immunohistochemistry, were mainly spindle-shaped cells resembling tissue macrophages outside KS lesions and some scattered cells (<5%) present within KS nodules.The demonstration of chemokine receptors in KS cells raises the possibility that recruitment of KS cells in response to locally produced chemotactic stimuli may be one of the events involved in the pathogenesis of Kaposi sarcoma.     

Spindle cell variant of epithelioid sarcoma: an easily misdiagnosed tumour

Epithelioid sarcoma is a histologically distinct soft tissue sarcoma of high grade malignancy. We report a case of epithelioid sarcoma in a young man who presented with multiple nodules over the left forearm, with bony invasion and pulmonary metastases. The histological features of the dermal tumour were those of a malignant spindle cell tumour with positive cytokeratin and vimentin staining and differed from the classical epithelioid sarcoma in its absence of typical necrobiotic nodular epithelioid pattern. It was the clinical presentation and the histology of the subcutaneous nodules that led to the final diagnosis of epithelioid sarcoma. This case illustrates a predominance of spindle cell pattern in the dermal tumour of epithelioid sarcoma, which has previously been reported as fibroma-like variant of epithelioid sarcoma.     

An "anaplastic" Kaposi's sarcoma mimicking a Stewart-Treves syndrome. A case report and a review of literature

Cutaneous angiosarcoma (AGS) developing in a lymphedematous arm, after lymphadenectomy in the context of breast cancer, is the definition of the classical Stewart-Treves syndrome. Like AGS, many tumors such as Kaposi's sarcoma (KS) could develop in chronic lymphedema. We describe the case of a 50-year-old woman who presented with several nodules on the left lymphedematous arm evocative of a Stewart-Treves syndrome, 2 years after a left mastectomy and a homolateral lymphadenectomy. The histological examination revealed an atypical vascular proliferation suggesting AGS, but endothelial atypical cells nuclei were strongly stained by herpes human virus 8 antibody. The final diagnosis was an "anaplastic" KS mimicking a Stewart-Treves syndrome. The total regression of the lesion was obtained by elastic contention and intradermic liposomal doxorubicin. "Anaplastic" KS is a rare histological form of nodular KS, which mimics a cutaneous AGS but classically expresses herpes human virus 8. It is essential to know about this entity, particularly in a lymphedematous arm, to avoid aggressive treatment such as amputation.     

Subcutaneous sarcoidosis--clinicopathological study of 10 cases

BACKGROUND: Subcutaneous sarcoidosis is a specific cutaneous lesion of sarcoidosis that is rarely reported. OBJECTIVE: Our purpose was to analyse the clinicopathological features of 10 patients with subcutaneous sarcoidosis and its relationship with the systemic features of the disease. PATIENTS AND METHODS: The patients with systemic sarcoidosis, diagnosed from 1974 to 2002 at a university hospital in Barcelona, Spain, who developed subcutaneous involvement, were included in the study. The diagnosis of systemic sarcoidosis was made according to conventional criteria. All the patients were monitored prospectively at the sarcoidosis clinic of the hospital. Skin biopsies were performed when granulomatous cutaneous involvement was suspected clinically. RESULTS: Granulomatous cutaneous involvement was demonstrated in 85 of 480 patients with systemic sarcoidosis. In 10 of these 85 patients subcutaneous sarcoidosis was diagnosed (11.8%). The lesions were most frequently located in the extremities, involving the forearms in nine patients. Indurated linear bands from the elbow to the hand were observed in five patients. In all of our patients the subcutaneous nodules appeared at the beginning of the disease. In six patients, the nodules remitted spontaneously in less than 2 years. In two cases foreign particles were detected under polarized light. CONCLUSIONS: Subcutaneous sarcoidosis is a quite uniform clinicopathological entity usually appearing at the beginning of the disease. It usually heralds forms of sarcoidosis with nonsevere systemic involvement and is not associated with chronic fibrotic disease.     

NECROBIOTIC XANTHOGRANULOMA*

Eight patients who developed unusual xanthomatous plaques with a tendency to ulceration and scarring and subcutaneous extension are described. Six patients had an IgG monoclonal serum paraprotein. Two patients had multiple myeloma and one patient had a plasmacytic lymphoma. Neutropenia, hypocomplementaemia and cryoglobulinaemia were variable features. Sixteen skin biopsies showed a consistent pattern of xanthogranulomatous nodules separated by zones of necrobiosis and an admixture of prominent giant cells. Lymphoid or plasma cell nodules may occur in the biopsies. Necrobiotic xanthogranuloma belongs to a range of xanthomas which has been associated with dysproteinaemia and lymphoreticular malignancy.     

Iatrogenic Mycobacterium abscessus infection: histopathology of 71 patients

The histopathology is described of 190 skin biopsies from 71 patients who presented during an epidemic of nodules and abscesses in the periumbilical region and buttocks, secondary to the application of xylocaine injections. Positive cultures for Mycobacterium abscessus were obtained from the specimens and from the xylocaine solution. The severe inflammatory lesions involved the dermis and the hypodermis and had three main histopathological patterns: (i) granulomatous nodular or diffuse inflammation with mixed granulomas in 57 (80%) of the cases; (ii) prevailing abscesses with mild granulomatous reaction in 28 (15%) of the biopsies; and (iii) deep dermal and subcutaneous granulomatous inflammation with no neutrophil component in three (4%) of cases. Bacilli were detected in 51 (27%) of the specimens, frequently forming small clumps at the centre of clear spaces or vacuoles and which were lipid-like structures in 156 (82%) of the abscesses or granulomas. This series represents one of the largest reported outbreaks caused by atypical mycobacteria and in which the source of infection was confirmed. The results emphasize the essential role of skin biopsies in determining the histopathological substrate, in helping to detect the atypical mycobacterial origin and in encouraging the practice of cultures for the identification of micro-organisms.     

Multiple mucinous and lipomatous variant of eccrine angiomatous hamartoma associated with spindle cell hemangioma: a novel collision tumor?

Eccrine angiomatous hamartoma (EAH) is a rare, benign condition characterized histologically by increased numbers of eccrine elements, as well as numerous capillary channels. In most cases, EAH arises as a single lesion; however, multiple variants have been reported. We report a 35-year-old female patient with multiple, sudoriparous, subcutaneous nodules on the right foot, which showed typical histopathological findings of EAH, and vascular components of the tumor consisted of thin-walled dilated vascular spaces intermixed with spindle cells and some histiocytoid endothelial cells representing spindle cell hemangioma (SCH). To our knowledge, the co-existence of EAH with SCH is a novel finding and not yet described.     

Expression of the fibroblast/macrophage marker 1B10 by spindle cells in Kaposi's sarcoma lesions and by Kaposi's sarcoma-derived tumor cells

BACKGROUND: Kaposi's sarcoma (KS) is a tumor whose ontogenic origin remains a matter of contention. KS tissues are characterized by predominant expression of endothelial markers, while KS-derived cell cultures are usually characterized by expression of mesenchymal non-endothelial cell markers. AIMS: In order to clarify the ontogenic origin of KS cells, we investigated the expression of the fibroblast/macrophage marker 1B10 in KS tissues (AIDS-associated KS, n = 9; classic KS, n = 6; iatrogenic KS, n = 6) and in KS-derived cell cultures. RESULTS: 1B10 was expressed by loosely distributed spindle-shaped cells in early 'patch-stage' KS and by a variable proportion of spindle cells in late 'plaque- and nodular-stage' KS. Using immunohistochemistry and immunoblot analysis, we found that, in vitro, reactivity for 1B10 was uniformly evidenced in fibroblasts and in KS-derived spindle cell cultures, irrespective of their histological or epidemiological setting. By contrast, vascular smooth muscle cells and endothelial cells were negative for 1B10. CONCLUSIONS: These results suggest that the KS spindle cells isolated in vitro may represent a particular subpopulation of the KS spindle cell compartment.     

Amebiasis cutis revisited

BACKGROUND: Amebiasis cutis (AC) is reported infrequently. This study assesses the clinicopathological spectrum, co-existent visceral involvement and impact of human immunodeficiency virus (HIV) co-infection on AC. METHODS: An 8-year prospective clinicopathological evaluation of patients with AC. RESULTS: Thirty-one biopsies of ulcers, fistulae, fissures, abscesses, polypoid and warty lesions in perianal, penile, scrotal, vulval, buttock, chest and abdominal wall sites were evaluated. Of these, 11 had a 'superficial' (superficial AC) and 20 a 'deep' (deep AC), histopathological pattern. Superficial AC showed predominant epidermal spongiosis, liquefactive necrosis, ulceration and fissures with hematophagous amebic trophozoites (HATs). Deep AC had confluent deep dermal and subcutaneous liquefactive, coagulative or suppurative necrosis and HATs. Seven biopsies showed vasculitis or thrombosis with luminal HATs. OUTCOME: Fourteen patients died; 9 had concomitant visceral amebiasis, 5 had other co-infections. Six who died were HIV seropositive, three were seronegative; all had deep AC. Of the 17 survivors, 11 (8 HIV positive) had superficial AC that healed with metronidazole treatment; the remaining 6 (one HIV seropositive) required additional surgical intervention. CONCLUSION: Deep AC is predictive of co-existent, contiguous visceral disease. The effective management, histopathological mimickers and diagnostic pitfalls of superficial and deep AC differ. The outcome in HIV-infected patients is dependent on co-existent systemic diseases.     

黏液炎性纤维母细胞性肉瘤一例

患者,女,69岁。左大腿红色斑块、结节伴瘙痒、疼痛2年。皮肤科查体：多个大小不一红色斑块、结节,质地较硬,基底深在,与周围组织界限不清,融合成片,大小约8.5 cm×11.0 cm,有压痛。组织病理学示：皮下梭形细胞肿瘤,奇异型核多见,局部病理性核分裂像,伴多量淋巴浆细胞浸润,可见间质粘液变性。免疫组化染色：Vimentin (+)、CD68(大部分+)、SMA(+),Ki-67指数(75%)。诊断：黏液炎性纤维母细胞性肉瘤。     

前臂结节性筋膜炎一例

患者,男,31岁。右前臂皮下结节5天,伴轻度压痛不适。高频超声检查示：表皮连续微隆起,真皮深层及皮下组织可见一类椭圆形低回声结节,边界清楚,体积约6.30 mm×6.23 mm×3.20 mm。皮损组织病理示：真皮及皮下组织大量梭形细胞呈束状排列,胶原纤维增生,间质黏液样变,伴少量淋巴细胞浸润及红细胞渗出。诊断：结节性筋膜炎。     

Characteristic appearance of spindle cell hemangiomatosis,often misdiagnosed as venous malformation: A retrospective study of 11 cases

Spindle cell hemangiomatosis is a benign multifocal vascular proliferation that mostly occurs in the distal extremities. It is a relatively rare disease and causes difficulties in clinical diagnosis and differential diagnosis of venous malformation. We retrospectively assessed the medical history, and clinical features, imaging features, pathological features and follow up of 11 patients diagnosed pathologically with spindle cell hemangiomatosis after surgery. There are two types of clinical appearances in spindle cell hemangiomatosis in the distal extremities: bleb-like nodules and varix-like nodules. Bleb-like nodules are mostly superficially located in the palm or interphalangeal joints with obvious hemorrhage in lesions and mainly composed of cavernous spaces rather than spindle cells. Varix-like nodules are located in the back of the hands or arms with normal skin color and mainly composed of solid areas of accumulated spindle cells. Surgery is the standard therapy for spindle cell hemangiomatosis, while sclerotherapy is invalid. Despite the tendency to develop new lesions, there are no residual lesions at the surgical site during follow up. Bleb-like nodules in the palm and interphalangeal joints are a typical clinical appearance in spindle cell hemangiomatosis which can help make clinical and a differential diagnosis of venous malformation. Surgery is the standard therapy for spindle cell hemangiomatosis with no residual lesions remaining at the surgical sites.     

Cutaneous sclerosing perineurioma of the digits: an uncommon soft-tissue neoplasm. Report of two cases with immunohistochemical analysis

BACKGROUND: Cutaneous sclerosing perineurioma is a recently characterized, uncommon tumor composed of perineurial cells, which exhibits immunoreactivity for epithelial membrane antigen (EMA). These lesions occur preferentially in children and young adults and usually develop as dermal or subcutaneous nodules in the hands or palms. METHODS: We report two cases of cutaneous sclerosing perineurioma in young patients without stigmata of neurofibromatosis. Histologically, these lesions were well-circumscribed masses and were characterized by a variable number of epithelioid and spindle cells with wavy nuclei end elongated cytoplasmic processes embedded in a dense collagen stroma. RESULTS: These cells showed focal whorling formation, demonstrated robust immunoreactivity for EMA and CD99, and were uniformly negative for S-100 protein, actin (HHF-35), CD34, cytokeratin AE1-3, and CD57. CONCLUSION: We comment on the differential diagnosis of fibrous cutaneous lesions based on immunohistochemistry.     

Obesity-associated lymphedematous mucinosis and stasis mucinosis

Cutaneous dermal mucinoses are a group of conditions characterized by abnormal deposition of mucin (hyaluronic acid and sulfated glycosaminoglycans) in the dermis. They can be classified either as localized or generalized forms and occur primarily or secondarily to systemic disorders. Obesity-associated lymphedematous mucinosis and pretibial stasis mucinosis are uncommon and relatively newly recognized disorders occurring in obese patients or in patients with venous insufficiency. Clinically, patients present with papules and nodules progressing to plaques and arising in an erythematous and edematous basis on the legs, especially the shins. Histopathologic examination indicates mucin deposition in the superficial portion of the dermis, angioplasia with an increase in small blood and/or lymphatic vessels, vertically running vessels, slight fibrosis, and no inflammation. Laboratory workup and histopathologic testing are helpful in differentiating these entities from pretibial myxedema (thyroid dermopathy), which represents the main differential diagnosis. Obesity-associated lymphedematous mucinosis and stasis mucinosis are not different entities but belong to a spectrum of mucinoses secondary to an increased body mass index, chronic lymphedema, and/or chronic venous insufficiency.     

Cutaneous mycobacterial spindle cell pseudotumor: a potential mimic of soft tissue neoplasms

A 55-year-old man with scleroderma treated with prednisone and etanercept presented with enlarging sporotrichoid nodules on the forearm. Microscopically, there were large circumscribed dermal and subcutaneous nodules of spindled and epithelioid cells, resembling a spindle cell neoplasm. Small foci of neutrophils were also present, and a subsequent Ziehl-Neelsen stain highlighted beaded acid-fast bacilli in the interstitium. Tissue culture demonstrated Mycobacterium chelonae. Cutaneous mycobacterial spindle cell pseudotumor is an exceedingly rare lesion, with only 6 previously reported cases. Although these included patients with autoimmune disease receiving immunosuppressive therapy, this is the first case reported in association with a tumor necrosis factor alpha inhibitor, etanercept. Furthermore, this represents the first mycobacterial spindle cell pseudotumor described in association with M. chelonae. Mycobacterial spindle cell pseudotumor should be considered in the differential diagnosis of cutaneous spindle cell proliferations, especially in immunocompromised patients.     

Histological characterization of regression in acquired immunodeficiency syndrome-related Kaposi's sarcoma

BACKGROUND: Kaposi's sarcoma (KS) is an angioproliferative lesion that may regress or progress. Progression is related to spindle cell proliferation and the expression of human herpes virus-8 latency genes, including latent nuclear antigen-1 (LNA-1), cyclin-D1, and bcl-2. KS regression has not been well characterized histologically. Therefore, this study was undertaken to characterize the histopathology of pharmacologically induced regressed cutaneous KS. METHODS: Skin punch biopsies from eight patients with acquired immunodeficiency syndrome (AIDS)-related KS, that regressed following chemotherapy with paclitaxel or the angiogenesis inhibitor Col-3, were investigated by light microscopy. Comparative immunophenotyping on pre- and post-treatment specimens for CD31, LNA-1, cyclin-D1, bcl-2, and CD117 (c-kit) was performed. RESULTS: Clinical and histologic features of regression were similar for paclitaxel and Col-3 treatment. On clinical examination, lesions flattened, became smaller, and lost their purple-red appearance, resulting in an orange-brown macule. Histological regression was divided into partial (n = 3) and complete (n = 5) regression. Partially regressed lesions had a significant reduction of spindle cells in the dermal interstitium, with residual spindle cells arranged around superficial and mid-dermal capillaries. Complete regression was characterized by an absence of detectable spindle cells, with a slight increase in capillaries of the superficial plexus. All regressed samples exhibited a prominent, superficial, perivascular, lymphocytic infiltrate and abundant dermal hemosiderin-laden macrophages. This clinicopathologic picture resembled the findings of pigmented purpura. CD31 staining correlated with the reduction of spindle cells. Regression was accompanied by a quantitative and qualitative decrease in LNA-1 and cyclin-D1 immunoreactivity, but no change in bcl-2 or c-kit expression. CONCLUSIONS: Pharmacologically induced regression of AIDS-related cutaneous KS is characterized by a complete loss or decrease of spindle cells, increased lymphocytes, and prominent dermal siderophage deposition. Without any prior knowledge of the history of KS regression following therapy, regressed KS lesions may be misdiagnosed clinically and histologically as pigmented purpuric dermatitis.     

Lymphedema: an immunologically vulnerable site for development of neoplasms

Lymphedema is the result of accumulation of protein-rich interstitial fluid (lymph stasis) caused by a failure of lymph drainage in the face of a normal capillary filtration. Whether the origin is congenital or acquired from infection, radiation, trauma, or surgery, chronic lymph stasis impairs local immune surveillance by disrupting trafficking of the immunocompetent cells in the lymphedematous district and stimulates vicarious angiogenesis by promoting development of a collateral lymphatic and hematic network in the lymphedematous district. When the local mechanisms of immune surveillance begin to fail, the lymphedematous region becomes an immunologically vulnerable area, predisposed to malignancy, chiefly vascular tumors such as Stewart-Treves syndrome and Kaposi's sarcoma, because of the continual angiogenic stimulus.