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.     

Extranodal interdigitating dendritic cell sarcoma presenting in the pleura: a case report

Interdigitating dendritic cell sarcoma (IDCS) is an extremely rare neoplasm arising from the antigen-presenting cells of the immune system. This disease usually involves the lymph nodes, and rarely, extranodal sites may be affected. The authors report a case of extranodal IDCS presenting in the pleura. A 32-yr-old man presented with progressive chest pain. Imaging studies showed diffuse pleural thickening with pleural effusion. Morphological and immunohistochemical analysis of an incisional biopsy of the pleura were consistent with a diagnosis of IDCS; tumor cells were positive for S100 and CD45, but negative for CD1a, CD21, CD35, B cell and T cell markers. The patient was administered chemotherapy, but died of progressive disease. Although its incidence is extremely rare, this case suggests that extranodal IDCS should be considered in the differential diagnosis of undifferentiated neoplasms and that immunohistochemical staining be performed using appropriate markers.     

The genetics of interdigitating dendritic cell sarcoma share some changes with Langerhans cell histiocytosis in select cases

Histiocytic disorders have been noted to have evidence of transdifferentiation; examples of cases with combinations of different lineages have been shown. In our index case, we identified interdigitating dendritic cell (IDC) differentiation in a case of Langerhans cell histiocytosis (LCH). Little is currently known about the genetics of IDC sarcoma (IDCS) because they are exceedingly rare. Using array comparative genomic hybridization (aCGH), we evaluated 4 cases of IDCS and compared them with our index case, as well as genetic abnormalities previously found in LCH. Four cases of paraffin-embedded samples of IDCS and 1 case of LCH with IDC differentiation were evaluated using aCGH. Array CGH results showed no abnormalities in a case of LCH with interdigitating cell differentiation. In 3 of 4 cases of IDCS, genetic abnormalities were identified; 1 case had no identifiable abnormalities. Interdigitating dendritic cell sarcoma case 1 had gains of 3q and 13q; IDCS case 2 had trisomy 12; IDCS case 3 had deletions of 7p, 12p, 16p, 18q, 19q, and 22q; and IDCS case 4 had no detectable abnormalities. Our index case, LCH with IDC differentiation, showed no abnormalities by aCGH. A number of LCH cases do not have detectable genetic abnormalities. In contrast, 3 of 4 cases of IDCS evaluated had identifiable abnormalities by aCGH. Furthermore, 2 of these shared abnormalities, albeit of large genetic regions, with published abnormalities seen in LCH. No recurrent abnormalities were identified in the IDCS cases. However, the possibility of a relationship between IDCS and LCH cannot be entirely excluded by these results.     

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.     

Dendritic cell sarcomas/tumours of the breast: report of two cases

Extranodal follicular dendritic cell sarcoma/tumours (FDCS/Ts) and interdigitating dendritic cell sarcoma/tumours (IDCS/Ts) are rare neoplasms. We present two cases of FDCS/T and IDCS/T of the breast. The FDCS/T case (case 1) presented in a 31-year-old woman and the IDCS/T case (case 2) in a 67-year-old woman who both showed a firm lump in the left breast. The FDCS/T lesion superficially appeared as an anaplastic carcinoma and the IDCS/T was reminiscent of a spindle cell sarcomatoid carcinoma. Nevertheless both lesions were negative for keratins while case 1 displayed neoplastic cells strongly positive for CD21, vimentin and focally for CD68 and S-100 protein. The tumour cells of case 2 were positive for S-100, CD68 and CD45. In breast, an unusual keratin negative tumour composed predominantly of spindle cells arranged in fascicles, storiform pattern or whorls with a lymphoid rich stroma should raise suspicion for FDCS/Ts or IDCS/Ts. The distinction from malignant tumours with similar features is discussed.     

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.     

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.     

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.     

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.     

Multifocal histiocytic sarcoma of the gastrointestinal tract

We report a rare case of extranodal histiocytic sarcoma with multifocal gastrointestinal tract involvement, which has not been documented in the literature so far. A diagnosis of interdigitating dendritic cell/ histiocytic sarcoma was made on the preoperative gastric biopsy. Computed tomography scan revealed multifocal, circumferential gastrointestinal wall thickening involving the stomach and jejunal loops. Patient underwent distal gastrectomy with extended D1 dissection and proximal jejunal resection with gastrojejunostomy. Immunohistochemistry profile of both the gastric and jejunal tumors was similar to the preoperative gastric biopsy. The histiocytic origin of the tumor was confirmed by positive reaction of the tumor cells for CD 163. She received four cycles of CHOP chemotherapy, and is free of disease three years, following surgery.     

Mediastinal follicular dendritic cell sarcoma involving bone marrow: a case report and review of the literature

We report a rare case of mediastinal follicular dendritic cell (FDC) sarcoma involving the bone marrow. The patient, a 46-year-old woman, had a clinically aggressive tumor in the anterior mediastinum that was initially diagnosed as a diffuse B-cell lymphoma. She received chemotherapy but showed no significant improvement. One year later, the patient presented at our institution with pelvic bone metastases. Biopsy specimens of the sacrum lesion and bone marrow were obtained. The diagnosis of FDC sarcoma was made based on histological examination and immunohistochemical findings, including strong positive staining of tumor cells for CD21, CD23, clusterin, and epidermal growth factor receptor (EGFR) and negative staining for CD20, CD30, CD45, CD1a, S-100, vimentin, and keratin cocktail. Histological examination and immunohistochemical studies of a previous biopsy of the mediastinal mass confirmed the diagnosis of mediastinal FDC sarcoma. The patient was treated with an appropriate chemotherapy regimen; 1 month later, follow-up bone marrow biopsy revealed no tumor cells. Although FDC sarcoma is considered a low-grade tumor, the tumor in the present case not only developed at an unusual location with bone metastasis but also involved bone marrow. To our knowledge, this is the first such case ever reported. This case also highlights the utility of EGFR as an immunohistochemical marker of dendritic cell tumors that could be used as a diagnostic tool and guide for choosing appropriate chemotherapy regimens.     

Mediastinal mixed dendritic cell sarcoma with hybrid features

This report describes a recurrent sarcoma involving the soft tissues of the posterior mediastinum with features of both follicular dendritic and interdigitating dendritic cells. Histologically, the tumour, which was a recurrent neoplasm 19 years after the initial removal, was composed of bland spindle shaped cells with interspersed inflammatory cells, predominantly lymphocytes. Immunohistochemically, the spindle cells were positive for S100 protein, CD45, CD68, and vimentin, but negative for CD21 and CD35 (markers of follicular dendritic cells). The immunophenotype was in keeping with interdigitating dendritic cells. However, ultrastructural examination demonstrated elongated cell processes joined by desmosome-like junctions-features in keeping with follicular dendritic cells. Follicular dendritic cell sarcoma and interdigitating dendritic cell sarcoma are rare neoplasms and a high index of suspicion is required to make a correct diagnosis. Immunohistochemistry and/or electron microscopy are required for a definitive diagnosis. This case shows that hybrid neoplasms can occur, which have features of both follicular and interdigitating dendritic cells.     

Synchronous interdigitating dendritic cell sarcoma and B-cell small lymphocytic lymphoma in a lymph node

A gradually enlarging axillary mass in a 79-year-old man was excised. The specimen was processed for light microscopy, immunohistochemical studies, and electron microscopy; gene rearrangement studies were also performed. A diagnosis of an interdigitating dendritic cell tumor of the lymph node and a B-cell small lymphocytic lymphoma occurring in the same anatomic location was made. We found that although rare cases of interdigitating dendritic cell tumor with an associated secondary malignancy have been described in the literature, to our knowledge, this is the first report of interdigitating dendritic cell tumor and synchronous neoplasm diagnosed at the same site. A possible relationship between the 2 disorders is also discussed.     

Interdigitating dendritic cell sarcoma presenting in the nasal region

Interdigitating dendritic cell sarcoma (IDCS) is an extremely rare neoplasm derived from antigen-presenting cells. IDCS displays aggressive behavior in a third of all cases, and the most common involvement is a solitary lymph node. We report the case of an 87-year-old woman with a right nasal mass. She underwent a wide local excision and right functional lymphadenectomy. Histopathological, immunohistochemical and molecular studies were consistent with the diagnosis of IDCS. Although it is extremely rare, this case suggests that extranodal IDCS should be considered in the differential diagnosis of undifferentiated fusiform neoplasms.     

A case of primary spindle cell variant of embryonal rhabdomyosarcoma of the prostate

We treated a rare case of spindle cell variant of embryonal rhabdomyosarcoma (RMS) of the prostate of a patient referred to our hospital for gross hematuria. Computed tomography and magnetic resonance imaging revealed a 4-cm-diameter mass with focal cystic change. Transurethral resection (TUR) of the prostate was performed to diagnosis and treat for complete urinary retention. Microscopically, the TUR specimen almost comprised a fascicular proliferation of spindle-shaped tumor cells, leading to the diagnosis of spindle cell sarcoma. The consequent total prostatectomy revealed the presence of rhabdomyoblasts in addition to the spindle cell proliferation. A MyoD1 p.L122R mutation was not detected in this tumor. The tumor recurred locally, with multiple metastatic lesions found soon after surgery. The patient received chemotherapy and radiation therapy but died 10 months after initial presentation. Although MyoD1 mutation is reported to define a clinically aggressive subset of embryonal RMS, spindle cell variant of embryonal RMS shows extremely adverse clinical outcomes irrespective of MyoD1 mutation.     

A case of interdigitating dendritic cell sarcoma/histiocytic sarcoma – a diagnostic pitfall

Interdigitating dendritic cell sarcoma (IDCS) and histiocytic sarcoma (HS) are two distinct rare hematolymphoid neoplasms, and HS derived from a likely pre-existing IDCS has never been reported in the English literature. Diagnosis of such entities in excised specimens is difficult, but becomes more difficult with the scant amount of materials obtained with fine needle aspiration (FNA) and core needle biopsy. Here we present an interesting and unique case of an IDCS located within a mesenteric mass, which was initially diagnosed as IDCS from the cytology of FNA and core needle biopsy specimens. After brief chemotherapy, the patient again developed abdominal pain, and a HS was diagnosed based on the excised segmental small intestinal specimen. While the exact relationship between the IDCS and HS cannot be ascertained, it is most likely that the HS is derived from the IDCS, although co-existing HS in addition to IDCS from the cytology specimen cannot be completely ruled out.     

Interdigitating dendritic cell sarcoma of salivary gland associated lymphoid tissue not associated with HHV-8 or EBV infection

Interdigitating dendritic cell sarcoma (IDCS) is an extremely rare malignancy derived from professional antigen presenting cells. This report describes a case of IDCS arising in the salivary gland associated lymphoid tissue of the parotid gland of a 51 year woman, presenting with a painless neck swelling. Histologically, sheets of S100(+)/Ccd68(+)/CD45(+)/CD34(-)/CD1a(-) spindle cells were surrounded with an inflammatory infiltrate with no evidence of B or T cell clonal proliferations. No evidence of either human herpesvirus 8 or Epstein-Barr virus could be detected by quantitative polymerase chain reaction in the tumour cells with serological evidence of previous Epstein-Barr virus infection. The patient remains well and disease free 24 months after presentation without specific treatment.     

Myxoid variant of follicular dendritic cell sarcoma arising in the breast

Follicular dendritic cell sarcoma is a malignant tumor of the follicular dendritic cell which can arise in extranodal sites. We present here a case arising as a mass in the breast of a 41-year-old woman. The tumor was composed of mildly pleomorphic spindly cells with pale ovoid nuclei and cell processes intimately admixed with mature lymphocytes. In much of the lesion the cells were dispersed in cords in a myxoid stroma, and elsewhere there were solid sheets. The neoplastic cells were immunoreactive for CD21, CD35, EMA, and S100 protein, but not for other lymphoid markers or cytokeratin. Electron microscopy showed interdigitating cytoplasmic processes with junctions but no external lamina. The differential diagnosis includes carcinoma, lymphomas, and a variety of myxoid sarcomas. The tumor recurred within a few months and displayed increased nuclear pleomorphism and lymphatic invasion but the patient appears free of disease 3 years after the further excision. This case extends the spectrum of follicular dendritic cell sarcoma in soft tissue sites.     

Interdigitating dendritic cell tumor with breast and cervical lymph-node involvement: a case report and review of the literature

Interdigitating dendritic cell tumor (IDCT) is an extremely rare malignancy. It occurs primarily in lymph nodes, but extranodal involvement has also been reported. A 38-year-old woman with IDCT with breast and cervical lymph-node involvement is reported in this paper. To our knowledge, this is the first case of IDCT originating from the breast. In the breast and lymph node, the tumor displayed diffuse sheets, fascicles and storiform growth pattern. It was composed of oval to spindle cells with pale to eosinophilic cytoplasm, ill-defined cell outlines, oval nuclei with vesicular chromatin and prominent eosinophilic nucleoli. Mitotic activity was three per ten high-power fields. The neoplastic cells were intermingled with small mature lymphocytes and plasma cells. Immunohistochemical studies showed that the tumor cells were strongly and diffusely positive for vimentin, CD68, S-100 protein, CD45/leukocyte common antigen and fascin and focally positive for lysozyme, alpha-1 antitrypsin and CD4. Ki-67 labeling index was 10%. The patient was treated with combined therapeutic approaches, including surgery, radiotherapy and chemotherapy. IDCT has the potential for an aggressive clinical course. However, 32 months after the initial diagnosis, the patient is still alive and being followed with a stable tumor burden.     

Gastric fibromyxoma, a distinct entity of pure fibroblastic tumor--an ultrastructural study

A rare case of fibromyxoma arising in the stomach of a 78-year-old male is presented. A well-defined, encapsulated tumor measuring 27x17x8 cm showed a lobular growth in the submucosa and muscularis propria, with no association with the propria mucosa. Histologically, the tumor was characterized by a diffuse, widely spaced proliferation of small spindle, stellate and round cells with dendritic outlines with fibromyxoid stroma. Neither cellular atypia nor mitotic figures were observed. Immunohistochemically, the tumor was positive for vimentin and CD34 but negative for CD117, alpha-smooth muscle actin, muscle actin, desmin and S-100 protein. Ultrastructurally, the tumor cells had irregularly shaped nuclei and cytoplasm containing moderate amounts of mitochondria, rough endoplasmic reticulum, subplasmalemmal densities, and short interdigitating processes. These findings indicated the fibroblastic nature of the tumor. The patient was well without recurrence 20 months after surgery. It is important to recognize that gastric fibromyxoma is a distinct entity and to include it in the differential diagnosis of gastric mesenchymal tumors, as the tumors show a different clinical behavior and as limited surgery with local excision is possible.