New insights inside the interdigitating dendritic cell sarcoma-pooled analysis and review of literature.

Interdigitating dendritic cell sarcoma is a rare haematological neoplasm with high debatable management protocols. The data extracted from 127 case reports published between 1981 and 2018 were analysed. The median age at diagnosis was 58 years with a male to female ratio of 1.65:1. The median OS and PFS of IDCS were 12 and 6 months, respectively, with a disease-specific mortality rate of 36.4%. Two-thirds of patients had a localised disease, while 30% had a disseminated form with 1-year mortality rates of 21.1% and 78.9%, respectively. Twenty per cent of cases were associated with other malignancies. Histologically, the proliferation of large spindle-shaped cells with fascicular growth was described in 84.3% of cases. Based on Cox-regression model, surgical resection was the only treatment modality linked to survival improvement with no recorded survival benefits of radiotherapy and chemotherapy. The 1-year mortality rates in resected and non-resected disease were 17.8% and 63.2%, respectively (P < 0.0001).

Primary cutaneous interdigitating dendritic cell sarcoma is a morphologic and phenotypic simulator of poorly differentiated metastatic melanoma: A report of 2 cases and review of the literature.

Interdigitating dendritic cell sarcoma (IDS) is a rare form of hematologic malignancy associated with an aggressive clinical course. Only 4 prior cases have been described as originating in the skin. We encountered two male patients ages 47 and 61years of age who presented with solitary cutaneous neoplasms diagnosed as IDS. Histologic exam showed a coalescing nested and multinodular proliferation of large pleomorphic epithelioid cells. In one case an initial diagnosis of melanoma was rendered. A recurrence 8months later was then interpreted as a primary cutaneous IDS. This patient died of widespread metastatic disease within 2years from his initial surgery. The other patient has recently undergone wide excision and radiation without any recurrence or metastatic disease during this short follow up time period. Both patients had a tumor exhibiting the same phenotypic profile comprising leukocyte common antigen, SOX10, S100, CD68, and CD163 positivity. In reviewing the 4 other reported cases, there is a similar older male predominance (mean age of 58years) although women affected were significantly younger (mean age of 28years); there was a predilection for the proximal extremities and the face. Patients treated with excision only developed recurrent disease with one patient subsequently dying of metastatic disease. Primary cutaneous IDS is a highly aggressive hematologic malignancy that has many overlapping features with poorly differentiated epithelioid and spindle cell melanoma including SOX10 staining. An aggressive treatment protocol at the beginning could optimize patient survival.

Primary Cutaneous Interdigitating Dendritic Cell Sarcoma: A Case Report and Review of the Literature.

Interdigitating dendritic cell sarcoma (IDCS) is a rare tumor of spindle to ovoid cells intermixed with lymphocytes and plasma cells. Primary cutaneous IDCS, with no nodal or other organ involvement is extremely rare, with less than 10 cases reported to date. Herein, the authors describe a case in which a 61-year-old man presented with scattered subcutaneous nodules on his left shoulder and right anterior thigh. A biopsy was performed, and the histopathologic findings revealed prominent, diffuse superficial, and deep dermal infiltration by an atypical epithelioid-shaped tumor forming sheets and cords infiltrating throughout the dermis. Immunohistochemical stains were strongly and diffusely positive for S100, CD45, CD68, and lysozyme, whereas CD21, CD23, CD3, CD20, CD30, CD34, and CD117 were all negative. The histologic and immunohistochemical findings were consistent with an IDCS. A positron emission tomography scan was negative for metastases, leading to the diagnosis of primary cutaneous IDCS. The patient was started on chemotherapy with gemcitabine and docetaxel, and was stable at 4 months follow-up. Our findings contribute to the limited existing literature on primary cutaneous IDCS. This is the first documented case in which chemotherapy with gemcitabine and docetaxel was implemented for treatment, helping to establish an optimal treatment protocol for clinical remission.

Spontaneous regression of interdigitating dendritic sarcoma in a patient with concurrent small lymphocytic lymphoma.

Interdigitating dendritic cell sarcoma (IDCS) is a rare tumour; only seven cases of IDCS associated with chronic lymphocytic leucaemia/small lymphocytic lymphoma (CLL/SLL) have been reported. We present the case of a 60-year-old man who presented with fevers, night sweats and significant unintentional weight loss. Investigations led to a diagnosis of synchronous SLL and IDCS. Subsequent fluorodeoxyglucose (FDG) positron emission tomography CT (PET-CT) imaging revealed an unusual clinical course with spontaneously resolving highly metabolic lesions. After 42 months of follow-up, the patient remains free of clinical symptoms and evidence of IDCS.

[Interdigitating dendritic cell sarcoma/tumor: a clinicopathologic study].

OBJECTIVE: To study the clinicopathologic characteristics and diagnostic criteria of interdigitating dendritic cell sarcoma/tumor (IDCS/T). METHODS: The clinical features, histologic findings and results of immunohistochemical study in six cases of IDCS/T were analyzed, with review of literature. RESULTS: The age of patients ranged from 20 to 68 years. The sites of involvement included lymph node, tonsil and soft tissue. Histologically, the tumor cells were arranged in sheets, fascicles or whorls and intimately admixed with abundant lymphocytes and plasma cells. They were oval to spindly in shape and contained pale eosinophilic cytoplasm, oval nuclei and distinct nucleoli.Immunohistochemical study showed that the tumor cells were positive for S-100 protein and CD68. CONCLUSIONS: IDCS/T is a rare malignant tumor with poor prognosis. It carries distinctive histologic pattern and immunophenotype. The entity needs to be distinguished from follicular dendritic cell sarcoma/tumor, anaplastic large cell lymphoma and other spindle cell sarcomas in occurring soft tissue.

Interdigitating dendritic cell tumor of the lymph node in the right submaxillary region: a case report and review of the literature.

Interdigitating dendritic cell tumor/sarcoma is an extremely rare neoplasm that mainly occurs in the lymph node, with only 51 cases reported in the literature to date. The authors report the case of a 41-year-old woman who presented with a 4-month history of a gradually enlarging painless mobile lymphadenopathy in the right submaxillary region. The lymph node mass was completely resected and was treated with 1 cycle of CHOP chemotherapy. After 10 months, she was alive with no evidence of disease. Because interdigitating dendritic cell sarcomas are rare and can show morphologic and immunohistochemical heterogeneity, correct diagnosis requires a high index of suspicion and complete pathological study.

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 tonsil.

Interdigitating dendritic cell sarcoma (IDCS) is an extremely rare malignancy derived from antigen-presenting cells, with 55 cases reported thus far. A standard treatment modality is still being debated. This report describes a 56-year-old female who presented with right tonsillar enlargement and right submandibular swelling for 6 months. Treatment with empiric antibiotics did not result in improvement of her symptoms. Fine needle aspiration of the tonsil revealed no malignant cells. Tonsillectomy was eventually performed due to persistent symptoms. Based on microscopic findings, immunohistochemical stains, and review of the literature, the present case was finally diagnosed as IDCS of the tonsil with cervical lymph node involvement. The patient received four cycles of CHOP (cyclophosphamide, doxorubicin, vincristine and prednisone) chemotherapy, and a clinically complete response was achieved followed by adjuvant radiation.

[Expression and function of VAP-33 in murine dendritic cell sarcoma].

OBJECTIVE: To elucidate the expression and function of VAP-33 gene in dendritic cell sarcoma (DCS) cell line. METHODS: The expression of VAP-33 in DCS cells was investigated by mass spectrum with immunoprecipitation membrane protein. DCS cells were treated with antigens in different dosages (150, 850, and 1500 microl) for 24, 48 and 72 h respectively. Cell morphology and phagocytosis activity of DCS cells were measured. Indirect immunofluorescence, confocal microscopy and Western blotting were used to study the distribution and expression changes of VAP-33. Moreover, DCS cells were treated with 0.5 mol/L insulin for 20 min first and followed by Western blotting to detect changes of VAP-33 and glucose transfer protein 4 (GLUT-4) in the total cellular protein, cytoplasmic protein and membrane protein. Confocal microscopy was used to document the expression and distribution changes of VAP-33 and GLUT-4 in DCS cells. RESULTS: VAP-33 expression was obtained at the cell membrane and in the cytoplasm of DCS cells. Upon antigen stimulation, DCS cells showed more active phagocytosis and morphologically became more elongated with branched protrusions. The expression of VAP-33 was decreased by the antigen stimulation. Upon the insulin stimulation, the expression of VAP-33 and GLUT-4 were increased and co-localized. CONCLUSIONS: VAP-33 expression in DCS originated from the dendritic cells (DC) seemed relating to the vesicle transportation during antigen processing in DC. Additionally, VAP-33 and GLUT-4 also take part in the glucose transportation in the cells.

Metastatic interdigitating dendritic cell sarcoma masquerading as a skin primary tumor: a case report and review of the literature.

We report an unusual case of a lymph node interdigitating dendritic cell sarcoma (IDCS), metastatic to skin, in a 73-year-old patient. The patient initially presented as having a primary skin tumor with lymph node metastasis. The metastatic IDCS was initially read as an atypical fibroxanthoma. However, the morphology seen on the lymph node excision, paired with immunohistochemistry and electron microscopy studies, was diagnostic for an IDCS. Additional immunohistochemistry was performed on the shave biopsy, confirming that the skin tumor was a metastasis. IDCS is a rare tumor that belongs to the histiocytic and dendritic cell group of tumors. Diagnosing this entity is difficult without the aid of ancillary testing such as immunohistochemistry and electron microscopy. In the workup of a spindle cell neoplasm, IDCS should be included in the differential diagnosis.

[Interdigitating dendritic cell sarcoma-a case report with literature review].

OBJECTIVE: To report a case of interdigitating dendritic cell sarcoma (IDCS). PATIENT MATERIAL: The patient was a 41-year-old man with a lymph node bulging in the left neck. Laboratory examination of peripheral blood and bone marrow was abnormal. The diagnosis of IDCS was made by immunohistochemistry and electron microscopy. Treatment of this patient with ABVD regimen (adriamycin, bleomycin, vinblastine, dacarbazine) resulted in obvious improvement, but did not control the tumor infiltration. CONCLUSION: IDCS has no distinctive clinical or pathohistological characteristics. Immunohistochemistry and electron microscopy are crucial in distinguishing it from other histiocytic/dendritic cell neoplasms. IDCS displays an aggressive behaviour, and the responses to chemotherapy are variable.