VOLUME

32



NUMBER

11



APRIL

10

2014

JOURNAL OF CLINICAL ONCOLOGY

Nasopharyngeal Hodgkin Lymphoma Case Report A 50-year-old woman presented to her primary care physician with right nasal congestion and right parotid swelling. Past medical history was not significant, and she was a current smoker with a 35 pack-year history. Symptoms did not respond to antibiotics or steroids, and she was referred to an otolaryngologist. Physical exam and nasal endoscopy demonstrated a swelling in the adenoid pad, more prominent on the right. A computed tomography (CT) scan confirmed the presence of a nasopharyngeal soft tissue density measuring 4.1 cm in diameter. A biopsy in the operating room subsequently revealed atypical lymphoid hyperplasia. There were scattered large atypical cells with prominent nuclei positive for CD30; focally positive for CD15; and negative for CD20, CD45, CD56, and Epstein-Barr virus (EBV; latent membrane protein 1). There were no clonal B cells on flow cytometry. This was felt to be suspicious for classical Hodgkin lymphoma. An adenoidectomy was subsequently performed. On repeat magnetic resonance imaging 3 months later, the mass had recurred, and she was referred to our institution’s department of head and neck surgical oncology. A nasal endoscopy with excision of nasopharyngeal mass was performed, and pathology demonstrated a nodular infiltrate of small lymphocytes with scattered classical Reed-Sternberg cells (arrow, Fig 1). The lymphoma cells were positive for CD15, and CD30 with a characteristic Golgi staining (Fig 2). They were negative for CD20, CD45, and EBV (latent membrane protein 1), and no clonal IGH gene arrangement was identified. CD3 stain highlights background T lymphocytes. This was felt to be consistent with lymphocyte-rich classical Hodgkin lymphoma of the nasopharynx.

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Additional work-up included [18]fluorodeoxyglucose positron emission tomography-CT (PET-CT) scan and a bone marrow biopsy. Bone marrow was negative for lymphoma, and PET-CT scan demonstrated fluorodeoxyglucose avidity only in the nasopharynx; standard uptake value maximum was 7.4, with no lymphadenopathy identified. She was therefore stage IEA. It was recommended that the patient receive two cycles of doxorubicin, bleomycin, vinblastine, and dacarbiazine (ABVD) chemotherapy followed by a restaging PET scan. However, pulmonary function tests obtained before initiating chemotherapy demonstrated a decreased diffusing capacity, at 61% of predicted level. She received two cycles of doxorubicin, vinblastine, and dacarbiazine. Restaging PET scan demonstrated a complete response. She was then referred for radiation therapy (RT) to the primary, and received 20 Gy in 10 fractions to the nasopharynx (2 Gy per fraction, five fractions per week) of involved field RT using intensity-modulated RT. Daily cone beam CT was done for treatment verification and image guidance. The lymph nodes in the neck were not included in the treatment volume. She tolerated treatment well without significant toxicity. She was without clinical evidence of disease recurrence at the last follow-up visit, 2 months post-RT. Discussion Waldeyer’s ring is a discontinuous ring of lymphoid tissue located in the pharynx, the major components of which include the palatine tonsils, adenoids, and lingual tonsils. Although non-Hodgkin lymphoma is known to involve Waldeyer’s ring as the primary site of disease in 5% to 10% of cases, this case represents an uncommon presentation of Hodgkin lymphoma.1 Hodgkin lymphoma of the nasopharynx is extremely rare and accounts for as few as 0.32% of all Hodgkin lymphoma cases.2 Overall, fewer than 90 cases of Hodgkin lymphoma involving the nasopharynx have been reported in the literature, and only 20 of those cases primarily involved the nasopharyngeal region as this case did.3 When primary nasopharyngeal Hodgkin lymphoma does occur, it is most common in young males and is usually stage I or stage II. Although the incidence is exceedingly rare, most case reports describe favorable prognosis. The treatment paradigm for this patient and others with early-stage Hodgkin lymphoma is upfront chemotherapy, most commonly with ABVD, with response re-evaluated after two cycles with CT or PET-CT scan. Complete responders proceed to involved field RT, whereas those with less than a complete response receive an additional two cycles of ABVD before RT.2

Matthew E. Johnson, Valentin G. Robu, and Aruna Turaka Fox Chase Cancer Center, Philadelphia, PA

AUTHORS’ DISCLOSURES OF POTENTIAL CONFLICTS OF INTEREST Fig 1. e40

© 2014 by American Society of Clinical Oncology

The author(s) indicated no potential conflicts of interest. Journal of Clinical Oncology, Vol 32, No 11 (April 10), 2014: pp e40-e41

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Diagnosis in Oncology

A

B

CD30

CD15

Fig 2.

REFERENCES 1. Ezzat AA, Ibrahim EM, El Weshi AN, et al: Localized non-Hodgkin’s lymphoma of Waldeyer’s ring: Clinical features, management, and prognosis of 130 adult patients. Head Neck 23:547-558, 2001 2. Anselmo AP, Cavalieri E, Cardarelli L, et al: Hodgkin’s disease of the nasopharynx: Diagnostic and therapeutic approach with a review of the literature.

Ann Hematol 81:514-516, 2002 3. Bensouda Y, El Hassani K, Ismaili N, et al: Primary nasopharyngeal Hodgkin’s disease: Case report and literature review. J Med Case Rep 4:116, 2010

DOI: 10.1200/JCO.2012.48.6183; published online ahead of print at www.jco.org on January 21, 2014

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© 2014 by American Society of Clinical Oncology

Downloaded from jco.ascopubs.org on October 4, 2014. For personal use only. No other uses without permission. Copyright © 2014 American Society of Clinical Oncology. All rights reserved.

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