Clinicopathological characteristics and differential diagnosis of interdigitating dendritic cell sarcoma / 中华肿瘤杂志

Objective@#To analyze the clinicopathological features and differential diagnosis of interdigitating dendritic cell sarcoma (IDCS).@*Methods@#The clinical pathological features of 7 IDCS were analyzed. Among them, the follow-up results of 6 cases were available.@*Results@#Among the 7 IDCS patients, 4 cases were male and 3 were female. The age of the patients ranged from 26 to 69 years.Three cases were originated from lymph nodes and 4 cases were originated from skin, stomach, adrenal gland and mesentery, respectively. Microscopically, the tumor cells presented as fascicular and storiform proliferation and infiltrated by lymphocytes. The tumor cells were short-spindle or ovoid, with indistinct border of cytoplasm. The immunohistochemistry results showed that tumor cells were S-100, Vim, CD68 and CD163 positive, and AE1/AE3, EMA, CD117, CD34, Desmin, SMA, CD1α, CD21, CD23, CD35, HMB45, Melan-A, MelanPan and ALK negative.The BRAF mutation and clonal rearrangement of T and B cells were not detected. Among the follow-up period of 7 IDCS patients, 3 occurred disease progressions.@*Conclusions@#IDCS is extremely rare with unique pathological features, and its lesion is not limited to the lymph node. The IDCS patients with extensive lesions may have worse prognose. The differential diagnosis of IDCS includes other histiocytic and dendritic cell neoplasms, malignant melanoma and soft tissue neoplasms.

Diagnosis and treatment progress of interdigitating dendritic cell sarcoma / 肿瘤研究与临床

Interdigitating dendritic cell sarcoma (IDCS) is a very rare hematopoietic tissue-derived dendritic cell malignancy. Because of the lack of specific clinical manifestations, pathological diagnosis is still the gold standard for the diagnosis of IDCS. However, there are no standard treatment programs of IDCS at present. Most reports in the literatures on single tumor mainly focus on surgery. And for patients with multiple metastases and relapses in the body, chemotherapy is the major treatment method. IDCS is characterized with high malignancy, rapid clinical progress and poor prognosis. This paper reviews the progress of IDCS diagnosis and treatment.

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.

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.

One case of interdigitating dendritic cell sarcoma concurrent with acute myelomonocytic leukemia and literatures review / 白血病·淋巴瘤

Objective To summarize and learn the biological properties and clinical features of interdigitating dendritic cell sarcomas (IDCS).Methods The first IDCS patient concurrent with acute myelomonocytic leukemia (AML-M4) described herein,to our knowledge,was studied and 62 IDCS cases reported previously in the literature were reviewed.Results The patient had a history of breast cancer as well as radiotherapy and chemotherapy of it,and the patient showed poor response to 4 cycles of sequential chemocherapy regimens.Based on the laboratory results,IDCS and AML-M4 in this patient were both of myelogenous origination.Furthermore,review of the 62 IDCS patients reported previously showed that as high as 17 ％ of the patients had malignant disease and received radiotherapy or chemotherapy before they got IDCS,and patients of this group had worse prognosis compared with counterpart.Conclusion IDCS has poor prognosis,and therapy-related type worse.Prophylactic measures and stringent screening of the second cancer in those who received chemoterapy or radiotherapy are appropriated and necessary.

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.