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Sustained remission after ABVD treatment for interdigitating dendritic cell sarcoma.

Helbig G, Wichary R, Pająk J, Budny M, Makowska M, Machura K, Kubeczko M, Kyrcz-Krzemień S - Contemp Oncol (Pozn) (2014)

View Article: PubMed Central - PubMed

Affiliation: Department of Haematology and Bone Marrow Transplantation, Silesian Medical University, Katowice, Poland.

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Interdigitating dendritic cell sarcoma (IDCS) is an extremely rare neoplastic proliferation of dendritic cells... EBV and HSV8 viral infections are thought to be involved in its pathogenesis, but understanding their role requires further studies... It was demonstrated that this neoplasm displays an aggressive clinical course, but a standard therapeutic approach has not been established so far... Different treatment modalities have been attempted, but they have mostly been ineffective... Computed tomography (CT) scan of the neck revealed bilaterally enlarged lymph nodes (size 4.5 cm × 3.5 cm)... Chest X-ray and CT scan of the abdomen did not detect any abnormalities... Initially, our patient presented with solitary cervical lymph node involvement, and such manifestation is the most common... It should be mentioned that the differential diagnosis of IDCS is broad and may include several neoplastic and non-neoplastic conditions... Radical surgery has been the mainstay treatment for patients with localised disease... Several cases were reported with successful outcome, but rapid relapses have also been observed... Disseminated IDCS requires more intensive therapeutic management, usually combined chemotherapy, but a standard approach is yet to be established... The schema primarily used for lymphoma have been tested, but the results were inconclusive... We present a patient who achieved a complete response after ABVD regimen, despite disseminated disease at presentation... A similar case has been reported in the literature, but the patient was older (a 44-year-old female) and the disease was disseminated below the diaphragm, including the small bowel and the liver... Resistance to chemotherapy was associated with dismal outcome, even when autologous stem cells transplantation (ASCT) was performed.

No MeSH data available.


Spindle shaped cells with spindled nuclei and eosinophilic cytoplasm. Original magnification 400×
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Figure 0001: Spindle shaped cells with spindled nuclei and eosinophilic cytoplasm. Original magnification 400×

Mentions: A 22-year-old, previously healthy female was referred to our Haematological Department with a diagnosis of IDCS. Eight months prior to admittance she accidentally noticed an enlarged cervical lymph node. No general symptoms were present at that time. The diagnostic work-up was started. Common reactive causes of lymphadenopathy were carefully excluded. Computed tomography (CT) scan of the neck revealed bilaterally enlarged lymph nodes (size 4.5 cm × 3.5 cm). Chest X-ray and CT scan of the abdomen did not detect any abnormalities. Positron emission tomography (PET) scan performed in February 2010 showed increased glucose uptake in the right cervical area. Histological examination of the excised lymph node was done and the study revealed sheets of spindled cells with a whorled pattern. The nuclei of these cells were spindled or ovoid (Fig. 1). There were some multinucleate cells. The cytoplasm was slightly eosinophilic. Among these spindled cells, there were numerous lymphocytes and plasma cells. Necrosis was absent. The neoplastic cells were strongly positive for S-100 protein (Fig. 2), fascin (Fig. 3) and vimentin. They weakly expressed CD68 (Fig. 4) and CD45 antigens. CD1a and CD30 were absent, whereas expression of CD4 was positive. A preliminary diagnosis of IDCS was proposed. Due to the rarity of this disease, the lymph node was examined by two other pathologists and the final diagnosis was established three months later. On admission to our Centre the physical examination was normal except the enlargement of the right cervical lymph node. Blood tests showed haemoglobin concentration of 12.8 g/dl, white blood cell (WBC) count of 6.8 × 109/l and platelet count of 245 × 109/l. Leukocyte differential was normal. Lactate dehydrogenase (LDH) activity and β2-microglobulin (B2M) levels were within normal ranges (140 IU/l and 1.6 ng/ml, respectively). Biochemistry panel was normal. Viral studies were negative for HBV, HCV, CMV and EBV. The test for HSV8 was not performed. Bone marrow trephine biopsy revealed no abnormalities. Repeated CT scan of the chest done in May 2010 demonstrated two “new” tumour masses; in the retrosternal area (size 10 cm × 1.5 cm) and in the aorto-pulmonary window (size 2 cm × 2 cm). The patient started chemotherapy consisting of an ABVD regimen (adriamycin 25 mg/m2, bleomycin 10 mg/m2, vinblastine 6 mg/m2, dacarbazine 375 mg/m2) on days 1 and 15 in a 28-day cycle. She was given 6 cycles of ABVD and showed good tolerance. PET scan was negative after the completion of chemotherapy. Currently, more than two years after ABVD, she remains in complete clinical remission.


Sustained remission after ABVD treatment for interdigitating dendritic cell sarcoma.

Helbig G, Wichary R, Pająk J, Budny M, Makowska M, Machura K, Kubeczko M, Kyrcz-Krzemień S - Contemp Oncol (Pozn) (2014)

Spindle shaped cells with spindled nuclei and eosinophilic cytoplasm. Original magnification 400×
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4507884&req=5

Figure 0001: Spindle shaped cells with spindled nuclei and eosinophilic cytoplasm. Original magnification 400×
Mentions: A 22-year-old, previously healthy female was referred to our Haematological Department with a diagnosis of IDCS. Eight months prior to admittance she accidentally noticed an enlarged cervical lymph node. No general symptoms were present at that time. The diagnostic work-up was started. Common reactive causes of lymphadenopathy were carefully excluded. Computed tomography (CT) scan of the neck revealed bilaterally enlarged lymph nodes (size 4.5 cm × 3.5 cm). Chest X-ray and CT scan of the abdomen did not detect any abnormalities. Positron emission tomography (PET) scan performed in February 2010 showed increased glucose uptake in the right cervical area. Histological examination of the excised lymph node was done and the study revealed sheets of spindled cells with a whorled pattern. The nuclei of these cells were spindled or ovoid (Fig. 1). There were some multinucleate cells. The cytoplasm was slightly eosinophilic. Among these spindled cells, there were numerous lymphocytes and plasma cells. Necrosis was absent. The neoplastic cells were strongly positive for S-100 protein (Fig. 2), fascin (Fig. 3) and vimentin. They weakly expressed CD68 (Fig. 4) and CD45 antigens. CD1a and CD30 were absent, whereas expression of CD4 was positive. A preliminary diagnosis of IDCS was proposed. Due to the rarity of this disease, the lymph node was examined by two other pathologists and the final diagnosis was established three months later. On admission to our Centre the physical examination was normal except the enlargement of the right cervical lymph node. Blood tests showed haemoglobin concentration of 12.8 g/dl, white blood cell (WBC) count of 6.8 × 109/l and platelet count of 245 × 109/l. Leukocyte differential was normal. Lactate dehydrogenase (LDH) activity and β2-microglobulin (B2M) levels were within normal ranges (140 IU/l and 1.6 ng/ml, respectively). Biochemistry panel was normal. Viral studies were negative for HBV, HCV, CMV and EBV. The test for HSV8 was not performed. Bone marrow trephine biopsy revealed no abnormalities. Repeated CT scan of the chest done in May 2010 demonstrated two “new” tumour masses; in the retrosternal area (size 10 cm × 1.5 cm) and in the aorto-pulmonary window (size 2 cm × 2 cm). The patient started chemotherapy consisting of an ABVD regimen (adriamycin 25 mg/m2, bleomycin 10 mg/m2, vinblastine 6 mg/m2, dacarbazine 375 mg/m2) on days 1 and 15 in a 28-day cycle. She was given 6 cycles of ABVD and showed good tolerance. PET scan was negative after the completion of chemotherapy. Currently, more than two years after ABVD, she remains in complete clinical remission.

View Article: PubMed Central - PubMed

Affiliation: Department of Haematology and Bone Marrow Transplantation, Silesian Medical University, Katowice, Poland.

AUTOMATICALLY GENERATED EXCERPT
Please rate it.

Interdigitating dendritic cell sarcoma (IDCS) is an extremely rare neoplastic proliferation of dendritic cells... EBV and HSV8 viral infections are thought to be involved in its pathogenesis, but understanding their role requires further studies... It was demonstrated that this neoplasm displays an aggressive clinical course, but a standard therapeutic approach has not been established so far... Different treatment modalities have been attempted, but they have mostly been ineffective... Computed tomography (CT) scan of the neck revealed bilaterally enlarged lymph nodes (size 4.5 cm × 3.5 cm)... Chest X-ray and CT scan of the abdomen did not detect any abnormalities... Initially, our patient presented with solitary cervical lymph node involvement, and such manifestation is the most common... It should be mentioned that the differential diagnosis of IDCS is broad and may include several neoplastic and non-neoplastic conditions... Radical surgery has been the mainstay treatment for patients with localised disease... Several cases were reported with successful outcome, but rapid relapses have also been observed... Disseminated IDCS requires more intensive therapeutic management, usually combined chemotherapy, but a standard approach is yet to be established... The schema primarily used for lymphoma have been tested, but the results were inconclusive... We present a patient who achieved a complete response after ABVD regimen, despite disseminated disease at presentation... A similar case has been reported in the literature, but the patient was older (a 44-year-old female) and the disease was disseminated below the diaphragm, including the small bowel and the liver... Resistance to chemotherapy was associated with dismal outcome, even when autologous stem cells transplantation (ASCT) was performed.

No MeSH data available.