Primary Hodgkin`s disease of the nasopharynx: a rare but

© Springer-Verlag 2002
Pathologica (2002) 94:314-316
CASO CLINICO
I. Abbes · K. Mrad · S. Sassi · M. Jellouli · L. Kochbati · M. Maalej · K. Ben Romdhane
Primary Hodgkin’s disease of the nasopharynx: a rare but bona fide disease
Il morbo di Hodgkin primario del nasofaringe: un riscontro raro ma riconosciuto
Abstract We report an uncommon case of Hodgkin’s disease confined to the nasopharynx. An isolated polypoid
mass of the nasopharynx was observed in a 41-year-old man
presenting with increasing bilateral nasal obstruction.
Histological study revealed a mixed cellularity type of
Hodgkin’s disease. Immunohistochemical analysis revealed
CD30 and LMP1 expression and a lack of reactivity to
CD15, CD3 and CD20. Serological tests excluded recent infection with Epstein-Barr virus. HD of nasopharynx is rare,
but has to be recognized as such in view of appropriate treatment.
Introduction
Hodgkin’s disease (HD) involving head and neck, in extranodal sites, is rare. Its primary location in the nasopharynx
is uncommon and rarely reported. Some of the previously reported cases were not confirmed immunohistochemically
and then may represent non-Hodgkin lymphomas, including
T cell lymphomas. We describe an unusual case of isolated
nasopharyngeal Hodgkin’s disease, which presented with
progressively increasing nasal obstruction.
Key words Hodgkin’s disease • Nasopharynx
Parole chiave Morbo di Hodgkin • Nasofaringe
Case report
I. Abbes • K. Mrad () • S. Sassi • K. Ben Romdhane
Histopathology Department, Salah Azaiez Institute,
10006 Bab Saadoun, Tunis, Tunisia
e-mail: [email protected]
Tel.: +216-71-577848
Fax: +216-71-574725
M. Jellouli
Service of Oto-Rhino-Laryngology,
Menzel Bourguiba Hospital, Menzel Bourguiba, Tunisia
L. Kochbati • M. Maalej
Service of Radiation Therapy,
Salah Azaïez Institute, Tunis, Tunisia
A 41-year-old man presented with a 6-month history of dyspnea, bilateral nasal obstruction and night snoring. Endoscopic examination of the nasal cavity revealed a smooth
budding mass arising from the nasopharynx. Computerized
tomographic scan of the nasopharynx revealed a soft tissue
mass of 2.5 cm extending to the right nasal fossa (Fig. 1).
This mass was further examined under general anesthesia
and then biopsied. Serological tests indicated previous
Epstein-Barr virus (EBV) infection with presence of type
IgG VCA and EBNA antibodies and absence of type IgM
VCA antibodies. Further scanning of neck, chest, abdomen
and pelvis was normal, with no evidence of lymphadenopathy or organomegaly.
Nasopharyngeal biopsy showed a dense cellular infiltrate
underlying the respiratory mucosa. This infiltrate was composed of tumoral cells scattered among polymorphous inflammatory cells (lymphocytes, plasma cells, histiocytes and
eosinophils). Tumoral cells presented abundant, slightly basophilic cytoplasm and enlarged mono- and bilobed nuclei
with prominent nucleoli and pale chromatin reminiscent of
Reed Sternberg cells (Fig. 2). Bone marrow core biopsy re-
I. Abbes et al.: Primary nasopharyngeal Hodgkin’s disease
315
expressed. These histological and immunohistochemical features were interpreted as classic HD, mixed cellularity type.
The patient was considered to have stage IA nasopharyngeal HD and was treated with radiotherapy alone. A total
dose of 40 Gy delivered on the Waldeyer’s ring and 36 Gy
delivered on bilateral cervical lymph node chains were fractioned over 1 month. To date, 12 months later, the patient is
well, with no evidence of recurrence or extension of the disease.
Discussion
Fig. 1 Computerized tomographic scan showing a soft tissue mass
of the nasopharynx extending to the right nasal fossa
Fig. 2 Tumor cells are large, with mono- or bilobated nuclei and
prominent nucleoli surrounded by a clear halo (HE, ×1000)
vealed no evidence of HD. A second biopsy of the nasopharynx, obtained 4 months later, revealed the same features together with a high number of Reed Sternberg cells
infiltrating the respiratory mucosa. Immunohistochemical
analysis, performed after microwave antigen retrieval, revealed para-nuclear and membrane expression of CD30 and
LMP 1 (Dako, diluted 1:50), while CD15, CD3, CD20, S100
protein, HMB45 and cytokeratin (Dako, prediluted) were not
Hodgkin’s disease involving Waldeyer’s ring, including nasopharynx, is rare. Most lymphomas at this site belong to the
non-Hodgkin’s lymphoma group [1]. Eavey and Goodman
reported only two cases of nasopharyngeal involvement in a
series of 500 cases of HD of the head and neck [2].
Bjorklund et al. [3] performed systematic biopsy of the nasopharynx in 45 patients with systemic HD and found nasopharyngeal involvement in 7 cases (16%). These authors
recommended that biopsy of the nasopharynx should be a
part of the staging procedure in HD. Primary occurrence of
HD in the nasopharynx is extremely rare; only 26 cases were
reported in the worldwide literature [4-9]. There is no clear
explanation why HD of the nasopharynx is such a rare condition although it is a reservoir of EBV and expansion of immunohistochemical techniques has not changed the low rate
of nasopharyngeal HD.
Diagnosis of isolated HD in the nasopharynx requires
exclusion of other lymphoproliferative disorders, especially
T-cell-rich large B cell and Lennert’s T cell lymphomas. In
the present case, EBV-induced atypical lymphoid hyperplasia was ruled out on serological profile (no evidence of recent EBV infection) and on immunohistochemical findings,
since atypical cells lack reactivity for both B and T cell lineage antigens. These results also excluded a T cell lymphoma and a T-cell-rich B cell lymphoma. Immunohistochemistry is determinant for the diagnosis. The required
panel is composed of five antibodies against leucocyte common antigen (LCA), B and T cell lineage antigens, Leu M1
(CD15) and Ber-H2 (CD30). The reactivity for CD30 and
LMP1 as well as the absence of expression of CD3 and
CD20 privileged the diagnosis of HD in this patient.
The treatment depends on the clinical stage and outcome
is similar to that of nodal HD.
This case illustrates the difficulty in the initial diagnosis
of HD primarily involving the nasopharynx. HD should be
considered in cases of atypical lymphoid proliferation, even
when confined to the nasopharynx. Immunophenotyping
studies using a restricted panel of 5 antibodies (LMP1, CD3,
CD20, CD15 and CD30) as well as EBV serological tests are
required.
316
I. Abbes et al.: Primary nasopharyngeal Hodgkin’s disease
Riassunto Riportiamo un raro caso di morbo di Hodgkin a
localizzazione nasofaringea. Una massa polipoide a livello
del nasofaringe è stata osservata in un uomo di 41 anni con
ostruzione nasale bilaterale. L’analisi istologica ha consentito una diagnosi di morbo di Hodgkin di natura cellulare
mista. L’analisi immunoistochimica ha evidenziato l’espressione di CD30 e LMP1 e assenza di reattività per CD15,
CD3 e CD20. Test sierologici hanno escluso recenti infezioni da Epstein-Barr virus. Il morbo di Hodgkin a livello del
nasofaringe è un riscontro raro, ma è comunque importante
un adeguato riconoscimento ai fini di un trattamento terapeutico appropriato.
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