Neoplasia de células dendríticas plasmocitoides blásticas, un reporte de caso / Blast plasmacytoid dendritic cell neoplasia, a case report

Introducción: La neoplasia de células dendríticas plasmocitoides blásticas (NCDPB) es una patología agresiva y representa menos del 1% de neoplasias hematológicas, se caracteriza por lesiones cutáneas nodulares violáceas sin evidencia de adenopatías en la mayoría de casos. Estudios observacionales demuestran que el Protocolo de quimioterapia Hyper-CVAD y la consolidación con tras-plante de células progenitoras hematopoyéticas se han asociado con una mayor supervivencia general. Caso Clínico: mujer de 82 años con antecedentes de Diabetes Mellitus tipo con cinco meses de lesiones hiperpigmentadas, elevadas, induradas, violáceas no dolorosas en mejilla, brazos, tórax anterior y posterior y piernas. Evolución: En citometría de flujo se determinó un fenotipo compatible con células patológicas (5.86%) con CD123++, HLADR+++, NG2++, CD56+++, CD4++, que sugiere una NCDPB. La biopsia de médula ósea presentó infiltración. PET CT posterior a terapia corticoide: no evidencia enfermedad tumoral macroscópica metabólicamente activa. Se inicia tratamiento con Dexametasona, con lo que las lesiones cutáneas disminuyeron en un 80%. Se inició Quimioterapia Protocolo CHOP like, ha recibido 6 ciclos hasta octubre del 2021, actualmente en remisión completa. Conclusión: En el presente caso el curso clínico de la NCDPB no fue agresivo hasta el momento del cierre del caso presentando disminución del 80% de las lesiones.

Introduction: blast plasmacytoid dendritic cell neoplasia (BPDCN) is an aggressive pathology and represents less than 1% of hematological neoplasms, it is characterized by violaceous nodular skin lesions without evidence of adenopathy in most cases. Observational studies show that the Hyper-CVAD chemotherapy protocol and consolidation with transplantation of hematopoietic progenitor cells have been associated with greater overall survival. Clinical case: a 82-year-old woman with a history of type Diabetes Mellitus with five months of hyperpigmented, raised, indurated, non-painful violaceous lesions on the cheek, arms, anterior and posterior thorax and legs. Evolution: Flow cytometry determined a phenotype compatible with pathological cells (5.86%) with CD123 ++, HLADR +++, NG2 ++, CD56 +++, CD4 ++, which suggests a BPDCN. The bone marrow biopsy showed infiltration. PET CT after corticosteroid therapy: there is no evidence of metabolically active macroscopic tumor disease. Dexamethasone treatment was started, with which skin lesions decreased by 80%. The CHOP-like Chemotherapy Protocol was started, she has received 6 cycles until October 2021, currently in complete remission. Conclusion: In the present case, the clinical course of NCDPB was not aggressive until the moment of closure of the case, presenting a decrease of 80% of the lesions.

Sarcoma diseminado de células dendríticas interdigitantes / Disseminated interdigitating dendritic cell sarcoma

Se describe el caso de una mujer de 70 años que consultó por dolor abdominal asociado a pérdida de peso y sudoración nocturna. En el examen físico se destacaban una masa abdominal comprendida entre el epigastrio y el flanco izquierdo, de unos 5 cm de diámetro, duro-elástica, móvil e indolora, y al menos tres adenopatías supraclaviculares bilaterales de 2 cm de diámetro, duras y adheridas a planos profundos. Se realizó una biopsia de la masa abdominal, con lo que se diagnosticó un sarcoma de células dendríticas interdigitantes. Se inició quimioterapia con el esquema CHOP (ciclofosfamida, doxorrubicina, vincristina y prednisona). Falleció luego de completado el primer ciclo del tratamiento, a los seis meses del diagnóstico.

A 70 year-old woman was admitted to our hospital with a 3-month history of abdominal pain, weight loss and night sweats. On physical examination, she presented with a 5 cm diameter abdominal mass extended from epigastrium to the left flank, and at least three bilateral supraclavicular adenopathies. A disseminated interdigitating dendritic cell sarcoma was diagnosed through a biopsy of the abdominal mass. After that, a CHOP regime (cyclophosphamide, doxorubicin, vincristine and prednisone) was iniciated. She died after completion of the first cycle of treatment, six months after diagnosis.

An unusual case of metachronous NK/T cell lymphoma and interdigitating dendritic cell sarcoma

No abstract available.

A Case of Disseminated Extranodal Interdigitating Dendritic Cell Sarcoma Arising from Parotid Gland / 고신대학교의과대학학술지

Interdigitating dendritic cell sarcoma (IDCS) is an extremely rare tumor derived from professional antigen presenting cell and primarily found in lymph nodes, with rarer case report about extranodal presentation of IDCS. A 71-yr-old man was admitted with progressively enlarging and painless mass in the right parotid area for 2 months. Computed tomography of the neck and chest revealed enhancing mass in right parotid gland, multiple lymphadenopathies around neck and mediastinum, and an osteolytic metastasis at thoracic spine. Morphological and immunohistochemical analysis of an excisional biopsy specimen from parotid mass were consistent with a diagnosis of IDCS. Palliative chemotherapy with 6 cycles of CHOP (cyclophosphamide, adriamycin, vincristine, and prednisolone) regimen and 2 cycles of ABVD (adriamycin, bleomycin, vinblastine, and dacarbazine) regimen plus radiotherapy on parotid mass failed in tumor reduction. We describe a rare case of disseminated extranodal IDCS arising from parotid gland.

Interdigitating dendritic cell sarcoma/tumor: a clinicopathologic study / 中华病理学杂志

<p><b>OBJECTIVE</b>To study the clinicopathologic characteristics and diagnostic criteria of interdigitating dendritic cell sarcoma/tumor (IDCS/T).</p><p><b>METHODS</b>The clinical features, histologic findings and results of immunohistochemical study in six cases of IDCS/T were analyzed, with review of literature.</p><p><b>RESULTS</b>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.</p><p><b>CONCLUSIONS</b>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.</p>

Successful treatment of disseminated interdigitating dendritic cell sarcoma with adriamycin, bleomycin, vinblastine, and dacarbazine chemotherapy / 대한혈액학회지

Interdigitating dendritic cell sarcoma (IDCS) is a very rare and aggressive neoplasm that arises from antigen presenting cells. IDCS usually involves lymph nodes; however, extra-nodal involvement has also been reported. Because a consistent standard therapy for IDCS has not been established to date, we report a case of the successful treatment of disseminated IDCS using ABVD chemotherapy (doxorubicin, bleomycin, vinblastine, and dacarbazine). A 64-year-old man was diagnosed with IDCS on the basis of immunohistochemical findings of a biopsy specimen of the inferior nasal concha. Immunohistochemical staining showed a positive reaction for CD68, leukocyte common antigen, and S-100 protein, but a negative reaction for CD34, CD1a, and CD21. Imaging studies showed cervical and axillary lymphadenopathies, subcutaneous nodules, and a soft tissue lesion in the nasal cavity. Treatment with the ABVD regimen resulted in complete remission after 8 cycles of chemotherapy.

Interdigitating dendritic cell sarcoma occured alone in axilla / 대한외과학회지

Interdigitating dendritic cell sarcoma (IDCS) is a very rare disease around the world and its prognosis is known to be aggressive. This reports a case diagnosed as IDCS of the axillary region treated in Soonchunhyang University Hospital. A 57-year-old female visited Soonchunhyang University Hospital with a left axillary mass. The mass was hard and fixed. Computed tomography observed a 7 cm lymph node at the left axilla, and core biopsy suspected sarcoma. In another study, there was no specific finding except the axillary lesion. Left axillary lymph node dissection (level I, II) was conducted and the pathologic report finally showed IDCS. The patient was treated with only radiotherapy and followed up without recurrence for 13 months up to now. IDCS is a very rare sarcoma that is hard to diagnose and progresses fast. Thus, treatment is very difficult. Proper treatment can be better established after more experiences.

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.

Follicular dendritic cell sarcoma: a clinicopathologic analysis of ten cases / 中华病理学杂志

<p><b>OBJECTIVE</b>To study the clinicopathologic features of follicular dendritic cell sarcoma (FDCS) and its differential diagnosis.</p><p><b>METHODS</b>Ten cases of FDCS were studied by light microscopy, immunohistochemistry and in-situ hybridization. The clinical features and follow-up information were analyzed.</p><p><b>RESULTS</b>Amongst the 10 cases of FDCS studied, the male-to-female ratio was 1:1. The mean age of the patients was 42 years. Six of them were located in cervical and peritoneal lymph nodes and four in extranodal sites (including tonsil, pelvic cavity, tail of pancreas and spleen). Histologically, the tumor cells had whorled, storiform or diffuse growth patterns. They were spindle in shape and contained syncytial eosinophilic cytoplasm, with round or oval nuclei, vesicular chromatin, distinct nucleoli and a variable number of mitotic figures. Multinucleated tumor giant cells and intranuclear pseudoinclusions were occasionally seen. There was a sprinkling of small lymphocytes and neutrophils within the tumor as well as in the perivascular region. Immunohistochemical study showed that the tumor cells were diffusely or focally positive for CD21, CD23, CD35 and D2-40, but negative for LCA, CD20, CD3, CD1a, HMB45 and CK. Some of them showed EMA, CD68 and S-100 reactivity. In-situ hybridization for Epstein-Barr virus-encoded RNA (EBER) showed positive signals in only one case (which was diagnosed as inflammatory pseudotumor-like FDCS). Of the 7 patients with follow-up information available (duration: 2 months to 39 months; mean: 14 months), 2 cases with paraneoplastic pemphigus died of pulmonary infection at 5 and 7 months respectively. The remaining 5 patients were alive and disease-free after surgical excision (+/- chemotherapy and radiotherapy).</p><p><b>CONCLUSIONS</b>FDCS is a rare low to intermediate-grade malignant tumor. Appropriate application of FDC markers, such as CD21, CD35 and D2-40, would be helpful for arriving at a correct diagnosis. Most cases are associated with good prognosis after surgical treatment, with or without chemotherapy and radiotherapy. Patients with paraneoplastic pemphigus carry a less favorable prognosis.</p>

Expression and function of VAP-33 in murine dendritic cell sarcoma / 中华病理学杂志

<p><b>OBJECTIVE</b>To elucidate the expression and function of VAP-33 gene in dendritic cell sarcoma (DCS) cell line.</p><p><b>METHODS</b>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.</p><p><b>RESULTS</b>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.</p><p><b>CONCLUSIONS</b>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.</p>

Interdigitating dendritic cell sarcoma/tumor: a study of 3 cases / 中华病理学杂志

<p><b>OBJECTIVE</b>To study the pathologic features, diagnosis and differential diagnosis of interdigitating dendritic cell sarcoma (IDCS).</p><p><b>METHODS</b>The clinical findings, morphologic features and immunophenotype of 3 cases of IDCS were investigated.</p><p><b>RESULTS</b>Gross examination showed that IDCS had a greyish-white to greyish-yellow cut surface. The site of occurrence included lung, spleen (with lymph node metastasis) and lymph node. Histologically, the tumor cells were arranged in nests, fascicles and whorls, with intimate admixture of many lymphocytes and plasma cells. They were oval to spindle in shape and contained pale eosinophilic cytoplasm, oval and sometimes grooved nuclei, small distinct nucleoli and ill-defined cell borders. Immunohistochemical study showed that the tumor cells expressed S-100 protein.</p><p><b>CONCLUSIONS</b>IDCS is a rare type of histiocytic and dendritic cell malignancy with distinctive morphologic findings. It needs to be distinguished from follicular dendritic cell sarcoma, inflammatory pseudotumor, Langerhans' cell histiocytosis, malignant melanoma, undifferentiated carcinoma and anaplastic large cell lymphoma. Immunohistochemical staining for S-100 protein is helpful in confirming the diagnosis.</p>

Dendritic cell sarcoma: 4 cases report with literature review / 中华血液学杂志

<p><b>OBJECTIVE</b>To describe the clinical and pathological features, treatment and prognosis of dendritic cell sarcoma (DCS).</p><p><b>METHODS</b>A group of DCS was described, including two cases of follicular dendritic cell sarcoma (FDCS), one each of interdigitating dendritic cell sarcoma (IDCS) and langerhans cell sarcoma (LCS). The related English literatures were reviewed.</p><p><b>RESULTS</b>Two patients with IDCS were a 19-year-old man and a 45-year-old woman respectively, both exhibited fever of unknown origin and painless lymphadenopathy. Pathological diagnosis of lymph node biopsy was FDCS with positive CD21 and CD35. Both patients achieved complete remission (CR) after 6 cycles of chemotherapy (CHOP: cyclophosphamide, epirubicin, vindesine, and prednisolone). However, the male patient relapsed 5 months later and another patient was still in CR at 5 months follow-up. One case of IDCS was a 42-year-old man, who manifested as paraneoplastic pemphigus. Biopsy of mediastinal lymph node demonstrated IDCS and immunohistochemistry showed positive S-100 staining. This patient died of pneumonia after two cycles of CHOP. One patient of LCS was a 54-years-old woman with fever, painless lymphadenopathy and diffused pulmonary nodules. The diagnosis of LCS was established after excisional biopsy was taken from inguinal lymph node. Positive staining of CD1a and S-100 was displayed by immunohistochemistry. Electron microscope examination confirmed the presence of Birbeck granule in tumor cells. Four cycles of chemotherapy (including ECHOP, FND) were administered, but the disease progressed.</p><p><b>CONCLUSION</b>DCS is a group of very rare sarcoma, FDCS, IDCS and LCS have different characteristic clinical features, immunophenotype and prognosis. The prognosis of most patients is poor.</p>

Interdigitating dendritic cell sarcoma-a case report with literature review / 中华血液学杂志

<p><b>OBJECTIVE</b>To report a case of interdigitating dendritic cell sarcoma (IDCS).</p><p><b>PATIENT MATERIAL</b>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.</p><p><b>CONCLUSION</b>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.</p>