HSV Pseudotumor of the Hypopharynx: A Diagnostic Challenge
Short running title: HSV Pseudotumor of the Hypopharynx
Mark I Knackstedt BS1, Syed HS Naqvi
MD1, Ron J Karni MD1
1Department of Otorhinolaryngology – Head & Neck
Surgery, McGovern Medical School at the University of Texas Health
Science Center Houston, Houston, Texas 77030
Corresponding Author:
Department of Otorhinolaryngology - Head and Neck Surgery - McGovern
Medical School
6400 Fannin St. St. 2700, Houston, TX 77030
Syed.Naqvi@uth.tmc.edu
(281) 925-8309
Keywords: herpes simplex virus; HIV; pharynx; pseudotumor; acyclovir
Key Clinical Message: HSV in the context of an immunosuppressed patient
can cause an inflammatory pseudotumor. These pseudotumors are rare
inflammatory reactions and can be mistaken for malignancy. Addressing
the cause of inflammation will resolve the lesion.
Please refer all correspondence, reprint requests, and proofs to Syed
Naqvi at the Department of Otorhinolaryngology - Head and Neck Surgery,
McGovern Medical School, 6400 Fannin St., Ste. 2700, Houston, TX 77030
– email: Syed.Naqvi@uth.tmc.edu
- phone - (281) 925-8309
Abstract:
A 50-year-old male presented with HIV and an ulcerative mass on the
hypopharynx. Initially thought to be malignant, multiple biopsies and
surgeries over 19 months did not resolve the lesion. Discovery of the
cytopathic effect on biopsy led to diagnosis of an HSV pseudotumor. The
lesion resolved with valacyclovir treatment.
Introduction:
The high rate of herpes simplex virus (HSV) seropositivity in the United
States makes it likely most surgeons and clinicians will deal with this
disease and its sequelae. The most common sequelae of HSV infection of
the oral cavity and pharynx is pharyngitis with vesicles and large,
painful, shallow ulcers.1 However, in cases of
immunosuppression, atypical presentations of HSV are common and new
sequelae are still being discovered. Patients with HSV are more likely
to transmit human immunodeficiency virus (HIV), and the two viruses are
frequently seen in conjunction. Recurrent ulcerative HSV is even
recognized as a possible early sign for immunosuppression. In these
coinfected individuals, rare hypertrophic pseudotumor forms have been
described in the genital region and oral cavity.1-4These rare diagnoses are often undiagnosed and unaddressed in this
population. We report a case of detrimental hypopharyngeal pseudotumor
development secondary to and HSV infection in the presence of long-term
HIV infection.
Case Report:
A 50-year-old African American male with a history of HIV, HSV, seizures
and smoking presented to the emergency room July 2020 for a 3-week
history of progressive dysphagia, nausea, and vomiting. His past medical
history was significant for a HAART controlled HIV infection diagnosed
in 2004, a partial penectomy due to a large necrotic HSV lesion which
had been recurring since 2000 and an ulcerative right hypopharyngeal
mass found in February 2019. In February 2019, he was found face down
beside his car with dyspnea and blood coming from the mouth. Rigid
laryngoscopy revealed an ulcer with heaped edges and granulation tissue
extending from the posterior pharyngeal wall down from the level of the
tongue base to the left hypopharynx and pyriform sinus. He was given a
tracheostomy and a biopsy of the ulcer was taken. Subsequent exploration
of the central neck for hypopharyngeal injury did not yield any results.
Pathology showed eosinophils and squamous epithelium on a background of
plasma cells without signs of malignancy. He passed a swallow study then
was decannulated and discharged to a rehabilitation center.
He was lost to follow up until July 2020 when he presented with
progressive dysphagia. A CT of the head and neck with contrast revealed
an extensive heterogeneously attenuating ulcerative mass filling the
supraglottic larynx with transglottic extension into the pyriform sinus
and hypopharynx. He was given a PEG and tracheostomy. A laryngoscopy at
the time revealed a large ulcerative mass on the right hypopharynx with
edema of the epiglottis and aryepiglottic folds. Several biopsies were
taken, and pathology found lymphocytes positive for CD45 and variably
positive for CD3 and CD20, but was negative for malignancy. He did not
follow up again until September 2020 where a foul smell was noted.
(Figure 1) At this time a neck CT with contrast revealed the
ulcerated mass had expanded to the point it was completely transglottic
with central nonnecrotic lymphadenopathy. A CD4+ count at the time was
259 and his virus load was undetectable. He returned to the OR for
another biopsy. Pathology found squamous mucosa with ulceration,
granulation tissue and extensive necrosis with sheets of eosinophils and
plasma cells. It was found the herpes virus cytopathic effect was
present and diagnosis was confirmed with immunohistochemical stain. The
patient was diagnosed with pseudotumor of the hypopharynx secondary to
HSV. He was then provided valacyclovir 1,000 mg q12 to help control the
ulcerative mass.
On follow up 4 weeks later, the mass had shrunken in size. This allowed
for increased visualization of the epiglottis. (Figures 2 and
3 ) The patient reported decreased dyspnea and cough with increased
energy.
Discussion
Though atypical presentations of HSV infections in the setting of
concomitant HIV infection have been described, no HSV induced
pseudotumors of the pharynx have been described in the literature. This
makes diagnosis of this case difficult as there are several other causes
of neck masses seen in patients infected with HIV.
In the setting of an HIV infected patient, pharyngeal masses can often
be caused by non-Hodgkin lymphoma, Kaposi sarcoma, p16 positive squamous
cell carcinoma or mycobacterium tuberculosis
infection.5 Biopsy for pharyngeal masses in HIV
positive patients is prudent and required as a primary diagnostic step.
If negative, the next best step is to consider possible opportunistic
infections.
Husak et al. were the first to describe an exophytic mass caused by HSV
acting as an opportunistic infection in an HIV positive patient. The
patient was obtaining treatment for genital complications of HSV when
the mass was noticed on his oral tongue. Pathology revealed eosinophilic
granulocytes, lymphocytes, and plasma cells without signs of squamous
cell carcinoma. He was given 300 mg brivudin daily to resolve the
infection.2 This has several characteristics similar
to our case and highlights the importance of the history of possible
opportunistic infections in HIV positive patients with atypical
presentations.
Tabaee et al. reported a case of a large exophytic mass formation of the
oral tongue status post cardiac transplant with immunosuppression.
Pathology was negative for carcinoma, but the squamous cells displayed
changes indicative of HSV infection. The patient was given 350 mg
acyclovir IV BID and then 400 mg of acyclovir po bid. The mass resolved
completely.1 This highlights the need to consider HSV
as an opportunistic infection in cases of immunosuppression outside HIV.
HSV infection of the genitalia causing large fungating masses are more
common than oral or pharyngeal HSV masses. History of genital mass
formation secondary to HSV may be an early sign of disseminated HSV
infection causing further complications.3 In the case
presented, the patient had an HSV-caused mass requiring removal several
years before presentation of the airway mass.
Conclusion
The formation of pseudotumors in the presence of concomitant HSV
infection and immunosuppression is a rare phenomenon with potentially
severe effects. Although this process has been described in the genital
region and, to a lesser extent, the oral cavity, it has not yet been
described in the pharynx. We recommend testing and treatment for HSV
infection in biopsy negative immunosuppressed patients with an
unexplained mass with valacyclovir.