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Poster display session

YO30 - Follicular dendritic cell sarcoma of the tonsil- a multimodality approach

Date

23 Nov 2019

Session

Poster display session

Topics

Tumour Site

Soft Tissue Sarcomas

Presenters

Rich Ericson King

Authors

R.E. King1, A. Villaruel2, J.P. Magno3, S.P. de Guzman4, L.I.G. Catedral1, K.A.M. Mondragon1, F.I.L. Ting1, R. Velasco, Jr.1, F.Y. Gracieux Jr.1

Author affiliations

  • 1 Division Of Medical Oncology, University of the Philippines - Philippine General Hospital, 1000 - Manila/PH
  • 2 Department Of Laboratories, Philippine General Hospital, 1000 - Manila/PH
  • 3 Department Of Otorhinolaryngology, University of the Philippines - Philippine General Hospital, 1000 - Manila/PH
  • 4 Division Of Radiation Oncology, University of the Philippines - Philippine General Hospital, 1000 - Manila/PH

Resources

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Abstract YO30

Case summary

A 53-year-old man presented with an 11-month history of a painless neck mass. On examination, he had a 10 x 5 cm mass at the left carotid triangle, and a fungating mass at the left tonsillar fossa, which on CT scan was 3.2 x 2.2 cm and invading into the parapharyngeal space.

Biopsy revealed an undifferentiated malignancy. Immunohistochemistry was negative for CK, LCA, EMA, synaptophysin, chromogranin and 34beta-E12, positive for vimentin and focally positive for S-100, favoring malignant melanoma. However, both HMB-45 and melan-A were negative, leading to a classification of undifferentiated sarcoma.

Chest and abdominal CT scan were negative for distant metastases. The tumor was deemed unresectable due to its close attachment to the great vessels, hence a sequential chemoradiation approach was pursued. He underwent six cycles of doxorubicin (60 mg/m2), resulting in 80% tumor regression. Unfortunately, he was lost to follow-up and was unable to undergo radiotherapy. He returned eight months later with re-enlargement of the neck mass to 6 x 5 cm. He remained free of distant metastases, and underwent two cycles of dacarbazine (250 mg/m2, day 1-5), which did not confer any response.

The indolent behavior of the tumor prompted pathological re-evaluation. Further staining showed diffuse positivity for CD21 and negative for CD1A and CD34, consistent with follicular dendritic cell sarcoma. The patient underwent three cycles of gemcitabine (1000 mg/m2, day 1 and 8) plus docetaxel (75 mg/m2, day 1), resulting in 50% tumor regression. This allowed dissection of level IB – V lymph nodes, which found disease in 10/23 nodes. He subsequently underwent adjuvant radiotherapy for the neck and primary radiotherapy for the tonsillar mass, with weekly gemcitabine (400 mg/m2) as a radiosensitizer. Evaluation three months post-treatment did not show any sign of disease progression.

Follicular dendritic cell sarcoma is very rare, accounting for <0.4% of soft tissue sarcomas. Only 60 cases of tonsillar occurrence have been reported in literature. Tonsillectomy is sufficient in most cases. For our patient, a trimodality approach done due to the presence of high risk features (R2 resection, LN involvement, tumor recurrence) provided good disease control.

Clinical trial identification

Editorial acknowledgement

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