HHV-8 is not associated with follicular dendritic cell tumours.

Follicular dendritic cell tumours are rare malignancies derived from the follicular dendritic cells of lymphoid follicles. These tumours have been associated with Epstein-Barr virus infections and with the hyaline vascular subtype of Castleman's disease. Because many examples of Castleman's disease have been associated with Kaposi's sarcoma associated herpes virus (HHV-8), this study uses polymerase chain reaction technology to examine five cases of follicular dendritic cell tumours for HHV-8. One of these cases had previously been documented to arise from pre-existing Castleman's disease. HHV-8 DNA was not detected in any of the follicular dendritic cell tumours examined, or in the original case of Castleman's disease. These findings suggest that HHV-8 plays no role in the aetiology of follicular dendritic cell tumours and the cause of this tumour remains obscure.     

Interdigitating dendritic cell sarcoma: Clinicopathologic study of 8 cases with review of the literature

To investigate the clinicopathologic features and differential diagnoses of interdigitating dendritic cell sarcoma (IDCS), the clinical, morphological and immunohistochemical features of eight cases of IDCS were collected and analyzed. Three patients were males and five were females, the mean age and the median age were 56.5 years and 57 years respectively. Clinically, the majority of cases involved lymph nodes. Microscopically, neoplastic cells were spindle or ovoid, forming fascicles or whorls. Every case had active mitosis figures. Immunohistochemically, these neoplastic cells were consistently positive for S100, but negative for CD21 and specific B-cell and T-cell associated antigens. Follow-up results were available in 7 cases, of which 5 cases of localized lesions survived, 2 cases died of organ involvement. Interdigitating dendritic cell sarcoma is an extremely rare neoplasm, with inferior prognosis and without standard treatment regimen. IDCS has similar but unique clinicopathologic features and the differential diagnoses include other histiocytic and dendritic cell neoplasms and malignant melanoma.     

指状树突细胞肉瘤/肿瘤的临床病理观察

目的 探讨指状树突细胞肉瘤/肿瘤(IDCS)的病理特征、诊断及鉴别诊断.方法 分析3例IDCS的临床表现,对标本进行病理形态和免疫组织化学(EnVision法)染色观察.结果 大体检查肿瘤呈灰白、灰黄色,质实.1例发生于左肺,1例原发于脾脏伴全身淋巴结累及,1例发生于淋巴结.光镜下肿瘤组织呈边界不甚清楚的巢片状,部分呈束状、旋涡状排列,周边较多淋巴细胞、浆细胞浸润.肿瘤细胞呈卵圆形或梭形,胞质透亮或轻度嗜酸性,核卵圆形,呈空泡状,可见不明显的小核仁,部分见有核沟.免疫组织化学肿瘤细胞表达S-100蛋白.结论 IDCS是一种罕见的组织细胞和树突细胞恶性肿瘤,有一定的病理学特征.应与滤泡树突细胞肉瘤、炎性假瘤、朗格汉斯细胞组织细胞增生症、恶性黑色素瘤、未分化癌及间变性大细胞淋巴瘤等鉴别.免疫组织化学S-100蛋白等标记对鉴别诊断有帮助.     

Epidemiology of human herpes virus 8 (HHV-8) or the herpes virus associated with Kaposi's sarcoma (KSHV)

Human herpesvirus 8 (HHV-8), also called Kaposi's sarcoma-associated herpesvirus (KSHV), is not a ubiquitous virus. In countries with a low viral seroprevalence (< 5% in adult general population) as the USA, Northern Europe and Asia, the infection concerns essentially homosexual men. In this latter population, the viral transmission seems to occur during sex. In endemic countries (HHV-8 seroprevalence between 10-70% in the adult general population) as in the Mediterranean basin (Italy, Greece), and Africa (East and Central Africa), men, women and children are infected. In these countries, HHV-8 seroprevalence increases with age and often reaches adult rates before the end of puberty. Viral transmission, in general endemic populations, seems to occur from mother to child and between sibs whereas heterosexual transmission appears to concern essentially groups at risk for sexual transmitted diseases. Saliva is a major reservoir of HHV-8.     

Interdigitating dendritic cell sarcoma. A report of four cases and review of the literature

To better define the clinical and pathologic features of interdigitating dendritic cell sarcoma (IDCS), we report 4 cases, including the first reported in the tonsil. There were 2 male and 2 female patients (mean age, 70 years). Sites of tumor included 1 case each in the right cervical lymph node, left axillary lymph node, right tonsil, and right inguinal lymph node. Histologically, all showed diffuse effacement of the lymphoid tissue by pleomorphic round to spindled cells with convoluted nuclei and abundant eosinophilic cytoplasm. All were immunoreactive for S-100, CD68, lysozyme, and vimentin. CD45 was positive in 3 cases and CD1a in 1 case. Fascin was positive in 3 cases. Other immunostains, including CD3, CD20, CD21, CD30, actin, cytokeratin, and HMB-45, were negative. Ultrastructurally, the tumor cells were elongated and showed indented nuclei, variable numbers of lysosomes, and interdigitating cytoplasmic processes. Follow-up was available for all cases. One patient died of widespread disease 2 months after diagnosis. One was alive with metastatic lung disease at 12 months. Two patients were disease free at 5 and 9 months.     

HHV-8 (KSHV) is not associated with bacillary angiomatosis.

AIMS: Bacillary angiomatosis is a rare pseudoneoplastic angioproliferative lesion occurring in patients with AIDS. This condition has been associated with Bartonella henselae and Bartonella quintana infections. Human herpesvirus 8 (HHV-8) is thought to be the causative agent of Kaposi's sarcoma, a vasoproliferative neoplasm, also commonly found in patients with AIDS. The presence of HHV-8 in a cohort of patients with bacillary angiomatosis was investigated. METHODS: Eight cutaneous cases of biopsy confirmed bacillary angiomatosis were assessed for HHV-8 using standard solution phase polymerase chain reaction (PCR). RESULTS: No case of bacillary angiomatosis harboured HHV-8 DNA. CONCLUSIONS: HHV-8 was not demonstrated in the lesions of bacillary angiomatosis and therefore does not appear to play a role in the pathogenesis of this pseudoneoplastic angioproliferative disorder. This finding might be useful in the distinction of bacillary angiomatosis from Kaposi's sarcoma, because lesions from patients with Kaposi's sarcoma almost always contains HHV-8 DNA.     

HHV-8 (KSHV) is not associated with bacillary angiomatosis.

AIMS: Bacillary angiomatosis is a rare pseudoneoplastic angioproliferative lesion occurring in patients with AIDS. This condition has been associated with Bartonella henselae and Bartonella quintana infections. Human herpesvirus 8 (HHV-8) is thought to be the causative agent of Kaposi's sarcoma, a vasoproliferative neoplasm, also commonly found in patients with AIDS. The presence of HHV-8 in a cohort of patients with bacillary angiomatosis was investigated. METHODS: Eight cutaneous cases of biopsy confirmed bacillary angiomatosis were assessed for HHV-8 using standard solution phase polymerase chain reaction (PCR). RESULTS: No case of bacillary angiomatosis harboured HHV-8 DNA. CONCLUSIONS: HHV-8 was not demonstrated in the lesions of bacillary angiomatosis and therefore does not appear to play a role in the pathogenesis of this pseudoneoplastic angioproliferative disorder. This finding might be useful in the distinction of bacillary angiomatosis from Kaposi's sarcoma, because lesions from patients with Kaposi's sarcoma almost always contains HHV-8 DNA.     

Interdigitating dendritic cell sarcoma: a report of four paediatric cases and review of the literature

AIMS: To report a series of four paediatric cases of interdigitating dendritic cell sarcoma (IDCS) and add to the known extranodal sites of occurrence for this tumour. Neoplasms derived from interdigitating dendritic cells are rare, with only 33 cases being reported in the literature (Medline search). These tumours usually occur in lymph nodes in the adult population. METHODS AND RESULTS: The patients were a 10-year-old girl with a large soft tissue mass bulging into the left chest, a 12-year-old girl with a right paraspinal mass, a 21-month-old boy with generalized lymphadenopathy and hepatosplenomegaly and a 6-year-old girl with a large bladder mass. Paraffin blocks and haematoxylin and eosin slides were available in all cases. In addition, immunohistochemistry and electron microscopy were performed. A diagnosis of IDCS was made in all cases. CONCLUSION: The diagnosis of IDCS can rarely be entertained on clinical information alone. Microscopically, there is a wide spectrum of features. Thus, immunohistochemistry and electron microscopy are crucial in making the diagnosis. The differential diagnosis includes inflammatory pseudotumour, follicular dendritic cell sarcoma, true histiocytic lymphoma, malignant Langerhans cell histiocytosis, anaplastic large-cell lymphoma, melanoma, and a range of sarcomas. IDCS displays aggressive behaviour and approximately half of the patients die of the disease.     

KSHV/HHV-8 associated Kaposi's sarcoma in lymph nodes concurrent with Epstein-Barr virus associated Hodgkin lymphoma

BACKGROUND: The unusual occurrence of a metastatic Kaposi's sarcoma (KS) in a lymph node affected by Hodgkin lymphoma (HL) was originally reported when knowledge of the specific virological features of these tumours was lacking. AIM: To re-evaluate this case by assessing whether the simultaneous presence of the two tumours was linked with common aetiopathogenetic factors. METHODS: The presence of EBV was investigated by in situ hybridisation, whereas KS associated herpesvirus (KSHV)/human herpesvirus 8 (HHV-8) was detected by immunohistochemistry. Both viruses were analysed in the case reported, in 30 lymph nodes from patients with classic HL, and in 22 skin biopsies from patients with KS. RESULTS: Consistent with the findings in the HL and KS cases analysed, in the case showing features of both HL and KS in the same lymph node, EBV was detectable only in Reed-Sternberg (RS) cells, but not in KS spindle cells, whereas KSHV/HHV-8 was detectable only in KS spindle cells, and not in RS cells. CONCLUSION: It is probable that the development of KS and HL was related to two independent aetiological cofactors-KSHV/HHV-8 and EBV, respectively-and that the occurrence of the two malignancies in the same patient was merely fortuitous.     

Assessment of a combined testing strategy for detection of antibodies to human herpesvirus 8 (HHV-8) in persons with Kaposi's sarcoma,persons with asymptomatic HHV-8 infection,and persons at low risk for HHV-8 infection

The performance of a human herpesvirus 8 (HHV-8) enzyme immunoassay (EIA) and selective subsequent use of an HHV-8 immunofluorescence assay (IFA) was tested in persons unlikely to be infected with HHV-8 and those who had HHV-8 detected in their saliva. The IFA was performed on samples within a range of EIA optical densities (0.15 to 0.35) where there was considerable overlap between HHV-8-infected and uninfected individuals. The sensitivity of the testing strategy was 88%, with a specificity of 97%.     

High prevalence of Human Herpes Virus 8 (HHV-8) in patients with Warthin's tumors of the salivary gland.

BACKGROUND: Warthin's tumor is a common benign neoplasm of the salivary gland. Human Herpes Virus 8 (HHV-8) is the etiologic agent for all forms of Kaposi's sarcoma (KS), and HHV-8 DNA is present in saliva, suggesting that non-sexual transmission is associated with latent infection of the salivary gland. OBJECTIVES: To provide insights into the HHV-8 cell tropism, the presence of HHV-8 was investigated in a series of Warthin's tumors of the salivary gland and corresponding adjacent normal tissue. STUDY DESIGN: Forty-three patients with Warthin's tumors (cystadenolymphoma) were tested for the presence of HHV-8 DNA, and corresponding adjacent normal tissue samples were obtained from 15 patients. DNA was extracted from the paraffin-embedded tissues. A nested polymerase chain reaction (PCR) assay was applied, and the positive samples were confirmed by direct sequencing. RESULTS: HHV-8 DNA was detected in 19 out of 43 (44%) salivary gland tumor samples. Among the 15 cases with paired samples, 9 were HHV-8-positive for both samples, 4 were HHV-8-negative for both samples while in two cases HHV-8 was detected only in the tumor specimens. CONCLUSIONS: HHV-8 is frequently detected in adenoid salivary neoplasms, suggesting a significant role of the virus in the etiopathogenesis of the disease. Larger studies are required to investigate the role of HHV-8 in the development or progression of Warthin's tumors.     

Interdigitating dendritic cell sarcoma of the duodenum with rapidly fatal course: a case report and review of the literature

Interdigitating dendritic cell sarcoma is an extremely rare malignancy derived from antigen-presenting cells. Dendritic cells constitute a heterogeneous group of cells, which includes Langerhans cells, dermal dendrocytes, follicular dendritic cells, and interdigitating dendritic cells present in lymphoid and nonlymphoid organs. We report the case of a 36-year-old woman who presented with epigastric pain, projectile vomiting, and significant weight loss. Upper gastrointestinal endoscopy showed a duodenal lesion; a biopsy of the lesion was taken and was diagnosed as sarcoma. She underwent a Whipple procedure. A final diagnosis of interdigitating dendritic cell sarcoma was made, with liver and peripancreatic lymph node involvement. The patient deteriorated rapidly and died 4 months later. Although interdigitating dendritic cell sarcoma of the duodenum is extremely rare, we think it should be included in the differential diagnosis of unusual spindle cell tumors with a rich lymphocytic infiltrate.     

HHV-8/KSHV is not associated with AIDS-related primary central nervous system lymphoma

Primary central nervous system lymphoma (PCNSL) is a major complication of the late stages of human immunodeficiency virus (HIV) disease. Epstein Barr virus (EBV) infection is the only genetic lesion consistently associated with this neoplasia. Recently, it has been proposed that the pathogenesis of AIDS-related PCNSL (AIDS-PCNSL) may be associated with infection by human herpesvirus-8/Kaposi's sarcoma associated herpesvirus (HHV-8/KSHV), although at present such association remains controversial. In order to conclusively assess the link between HHV-8/KSHV infection and AIDS-PCNSL, we performed a comprehensive study based on multiple molecular assays on cerebral tissues and cerebrospinal fluid (CSF) as well as specific immunologic assays on patients' sera. A well characterized panel of 33 Italian patients with AIDS-PCNSL and 13 controls with other HIV-related brain focal diseases from the same geographical area was analyzed. No signs of HHV-8/KSHV infection were detected in cerebral tissues by single-step PCR. Cerebral tissues of all AIDS-PCNSL scored negative for HHV-8/KSHV DNA sequences also by nested PCR, with the exception of one single patient who was simultaneously affected by Kaposi's sarcoma. All CSF samples analyzed were consistently devoid of HHV-8/KSHV sequences by molecular assays. By serologic assays, detecting both latent and lytic HHV-8/KSHV antigens, a specific immunoreactivity was observed in 16/33 (48%) AIDS-PCNSL and in 6/13 (46%) controls (P = 0.88). A significant correlation with HHV-8/KSHV serum reactivity was seen with a homosexual route of HIV transmission (P = 0.018), but not with the presence of AIDS-PCNSL. The results of our analysis conclusively assess that HHV-8/KSHV infection is not a feature of AIDS-PCNSL.     

Interdigitating dendritic cell sarcoma of lymph node mimicking granuloma: a case report and review of the literature

Interdigitating dendritic cell sarcoma (IDCS), is an extremely rare neoplasm. We report a case of a 77-year-old man presented with gradual lymph nodes enlargement in inguina, neck and axilla for 6 months. Biopsy revealed that part of the lymph node was replaced by several large granuloma-like nodules composed of mild atypical tumor cells, resembling epithelioid cells. Mitotic figures were hardly found. Immunohistochemistry showed that tumor cells were positive for S-100, CD68 and CD45. Ki-67 labeling index was 5%. To the best of our knowledge, this is the first case of IDCS showing granuloma-like growth pattern with mild atypical tumor cells.     

Cell-associated HIV-1 infection of salivary gland epithelial cell lines

In this study we investigated the potential of cell-associated primary isolates of HIV-1 to infect epithelial cells of the oral cavity, using two cell lines originating in the salivary gland, HSY and HSG. Neither of these cell lines expresses surface CD4; however, both express the alternative epithelial receptor galactosylceramide and the coreceptor CXCR4. The ability of these cell lines to be infected via cell-associated infection was studied using a lab-adapted strain and primary isolates of HIV-1. Both HIV-1 phenotypes were able to infect these cell lines. The ligand for CXCR4 was able to block infection of both cell lines, whereas the ligands for CCR5 were able to block infection in HSY only, indicating that these receptors were utilized for entry into the epithelial cells. These findings demonstrate that epithelial cells of the oral cavity can be productively infected with HIV-1 by cell-borne virus in the absence of CD4.     

滤泡树突状细胞肉瘤的临床病理观察

目的 探讨和分析滤泡树突状细胞肉瘤(FDCS)的临床病理特点及鉴别诊断.方法 应用组织学、免疫组织化学(EnVision法)标记及EBER原位杂交,对8例FDCS进行临床和组织病理学分析,并复习相关文献.结果 8例FDCS中男性5例,女性3例,平均年龄50岁.发生部位淋巴结4例,扁桃体、鼻咽部、肝、脾各1例.组织学:瘤组织呈席纹状、束状、弥漫性、旋涡状或结节状,肿瘤细胞呈合体样,境界不清,胞质较丰富,均质嗜伊红或细颗粒状,核呈卵圆形、短梭形或圆形,染色质稀疏或呈空泡状、点彩状,核仁明显,核分裂象多少不等,肿瘤细胞间见有散在淋巴细胞混杂.间质内可见假血管腔及血管周围淋巴鞘现象.其中肝脏1例以大量小淋巴细胞弥漫分布为背景,梭形或卵圆形的瘤细胞散在分布其中,瘤细胞核染色质细腻,部分区域细胞有轻度异形,核不规则、空泡状,有核仁.免疫组织化学瘤细胞均表达CD21、CD35、clusterin,部分表达CD68、上皮细胞膜抗原、S-100及内皮生长因子受体,Ki-67不同程度表达.EBER两例表达.结论 FDCS是一种非常少见的恶性肿瘤,易复发和转移,明确诊断需要结合病理形态学和免疫表型.     

Interdigitating dendritic cell sarcoma of the ileum recurred in multiple lymph nodes and duodenum three years after operation without chemotherapy

Neoplasms derived from interdigitating dendritic cell are extremely rare. Here we describe a case of a 47-year-old man with interdigitating dendritic cell sarcoma (IDCS) in the ileum. He was admitted to a hospital due to ileus. The ileal tumor, measuring 2 cm, was detected and resected with regional lymphadenectomy. At that time, a pathologic diagnosis of malignant peripheral nerve sheath tumor was made. The patient, who was not treated with chemotherapy, showed no signs of recurrence. After three years, we detected cervical lymphadenopathy and multiple duodenal masses in the patient in our hospital. Oval to spindle-shaped atypical cells, which resembled ileal tumor cells, infiltrated into the lymph node and duodenum. Immunohistochemical staining of these three lesions revealed positivity of S100 protein and several macrophage-related antigens. Based on the histologic and immunohistochemical analysis, the histopathologic diagnosis of IDCS was confirmed. To our knowledge, five cases of IDCS arising in the intestinal tract have been reported to date, and only one case, treated with both surgery and chemotherapy, led to remission. This is the first case that has a comparatively favorable prognosis without chemotherapy after surgery.     

Distribution of tissue markers in acinic cell carcinomas of salivary gland.

Eight cases of acinic cell carcinoma of the salivary glands were histologically reclassified and their immunohistochemical expression and distribution for various tissue antigens were examined. The epithelial elements were divided into tubuloglandular components, microcystic patterns and solid nests. The authors' results indicated the following: 1) The duct luminal cells of tubuloglandular components have distinct epithelial features with cytokeratin (KL 1), alpha 1-antichymotrypsin (alpha 1-ACT), transferrin, lactoferrin, IgA, and carcinoembryonic antigen (CEA) positivity. 2) The cyst-lining cells of microcystic pattern expressed immunophenotypes similar to those of the duct luminal cells. 3) The acinic cells in solid nests had positive results for KL 1, alpha 1-antitrypsin (alpha 1-AT), transferrin, lactoferrin and vasoactive intestinal polypeptide (VIP). 4) The clear cells in solid areas had positive results for KL 1, alpha 1-AT, transferrin and VIP. Both the clear cells and the neoplastic acinic cells showed a rather similar pattern of immunoreactivity. Therefore, the clear cells may transform from the neoplastic acinic cells. 5) Secretory products in tubuloglandular and microcystic patterns had positive results for alpha 1-ACT, lactoferrin, IgA and CEA. 6) The basement membrane-like material between the neoplastic islands has distinct positivity for alpha 1-AT. The result suggests that alpha 1-AT is a useful marker of basement membrane-like material.