Tonsillar Kaposi's Sarcoma: Case Report and Review of Literature
Shivam Saxena, Joseph Curry, Madalina Tuluc
Abstract
Kaposi's sarcoma is a tumor of vascular connective tissue that has been linked to
human herpesvirus 8 (HHV-8) infection. Kaposi's sarcoma of the tonsil is a rare presentation that
has been described in both HIV-positive and HIV-negative patients. Here we
describe a patient with enlarged tonsils revealing bilateral tonsillar Kaposi's
sarcoma. The patient presented with sore throat, facial pain, and cervical
lymphadenopathy. He underwent bilateral tonsillectomy and was diagnosed with
Kaposi's sarcoma. Immunohistochemical stains confirmed the presence of HHV-8.
Due to rare involvement of the tonsils, a review of the literature was
performed to describe common findings in patients with tonsillar Kaposi's
sarcoma. We conclude that common symptoms associated with tonsillar Kaposi's
sarcoma are a mass in the tonsillar fossa accompanied by sore throat, dysphagia
and cervical lymphadenopathy. We encourage physicians to include Kaposi sarcoma
in the differential diagnosis of tonsillar masses, especially in high risk patients and to be aware of symptoms associated with its
presentation.
Keywords: Kaposi’s, Sarcoma; Tonsils; Human Herpesvirus; HIV; High Risk.
Shivam Saxena, Joseph Curry, Madalina
Tuluc. Tonsillar Kaposi's Sarcoma:
Case Report and Review of Literature. International Journal of Oral &
Maxillofacial Pathology; 2013:4(1):00-00. ©International Journal of Oral and Maxillofacial
Pathology. Published by Publishing Division, Celesta Software
Private Limited. All Rights Reserved.
Received on: 28/10/2012
Accepted on: 31/05/2013
Introduction
Kaposi's sarcoma is a vascular neoplasm associated with human herpes
virus 8 (HHV-8) infections. The disease has four subtypes: classic (affects elderly men of Mediterranean and Eastern European descent), endemic (commonly found in Africa), immunosuppression-associated (especially posttransplant patients) and epidemic (seen in AIDS patients).1 Kaposi's sarcoma displays a large sex disparity with men having an incidence ratio rate 29 times greater than women.2 Classically, Kaposi's sarcoma presents with pigmented purple-blue skin lesions on the extremities that may progress to viscera and involve the gastrointestinal tract.1 Although Kaposi's sarcoma affects the head and neck in 40-67% of cases and usually involves the hard palate, there have been very few reports of primary Kaposi's sarcoma in the tonsil.3,4 Here, we describe a case of tonsillar Kaposi's sarcoma and review the literature to emphasize the importance of this location for the primary development of Kaposi's sarcoma.
Case Report
A 31-year-old male (of unknown HIV status) presented to the emergency
department with sore throat and moderate facial pain of
two day duration. Physical exam revealed tonsillar erythema, tonsillar exudate, and cervical lymphadenopathy. The patient was discharged with a prescription for Penicillin V but returned five weeks later with similar symptoms. Throat cultures were performed and revealed S. Aureus and the patient was discharged with Clindamycin. Patient followed up with the ENT department and local examination showed a large, irregular right tonsil and slight enlargement of the left tonsil. Adenoids also appeared abnormal and bilateral tonsillectomy with biopsy of adenoids was performed. The histologic exam revealed mitotically active spindle cell proliferation replacing the tonsillar parenchyma. Numerous extravasated erythrocytes and few slit-like vascular spaces were also present (Fig.1, Fig.2). The above histologic features were similar in the tonsils and the adenoid biopsy. Immunohistochemical stains were performed and the spindle cells were positive for CD34 and HHV8 (Fig.3). Histologic characteristics corroborated with immunohistochemistry profile confirmed Kaposi sarcoma.
Discussion
One of the first cases of tonsillar
Kaposi’s sarcoma was reported by Raikundalia5 in 1973, who wrote
about a 38 year old female presenting with painless swelling in the oral cavity along with dysphagia, dry cough, dyspnea, and blood tinged sputum. Upon examination, a large, firm, ulcerated mass was found protruding from the left tonsil. Patient also presented with cutaneous lesions and inguinal lymphadenopathy. Their
histologic findings confirmed Kaposi’s sarcoma.
Fig 1: Biopsy of the tonsils revealed a vascular neoplasm composed of mitotically active spindle cells and numerous extravasated erythrocytes.
(H&E, 10x)
Fig 2:
This image demonstrates slit-like vascular channels and extravasated erythrocytes
characteristic of Kaposi’s sarcoma. (H&E, 20x)
Fig 3: An immunohistochemical stain for HHV-8 shows positive nuclear staining in lesional
cells. (20x)
A 1998 article by Chetty6 et
al. reported two young HIV-positive male patients, whose initial presentation of Kaposi's sarcoma was tonsillar enlargement. The first patient, a 19-year-old,
presented with sore throat, enlarged hemorrhagic left tonsil, and abnormal enlargement of the adenoids. The second patient, a 25-year-old man, presented with dysphagia, odynophagia, and a hemorrhagic right tonsil. Both patients had cervical lymphadenopathy and no cutaneous involvement was noticed in either patient. Biopsy of each patient's tonsils revealed pathognomic Kaposi's sarcoma histology: spindle cells with slit-like vascular spaces filled with extravasated erythrocytes. The tumor cells were positive for endothelial cell markers CD31 and CD34.7
Kaposi's sarcoma is the most common malignancy in AIDS patients and presents infrequently in immunocompetent patients.1 Interestingly, a 2008 case report8 reported two related HIV-negative patients with evidence of Kaposi's sarcoma when worked up for a tonsillar mass. The
first patient was a 49-year-old male with a slow growing right tonsillar mass who
denied any other symptoms. His social history was negative for smoking, alcohol, and drug use. Physical exam of the oropharynx showed a 1cm smooth non-ulcerated mass. Fine needle aspiration of a mobile right level II lymph node in the neck showed spindle cells characteristic of Kaposi's sarcoma. Microscopic examination of the tonsils following bilateral tonsillectomy revealed the same diagnosis. Post-surgically, patient was followed for three years and remained in good health.
The second patient was a 34-year old male and the nephew of the
previous patient. He presented with a long history of tonsillitis and halitosis. Similar to the
first patient, he denied any constitutional symptoms. Medical history included asthma and a ten pack-year history of smoking that had ceased ten years prior to this presentation. Oropharyngeal examination was significant for a two centimeter defined mass at the base of the left tonsil, tonsillolithiasis, and bilateral enlargement of the tonsils. Although no lymphadenopathy was noted at the time of presentation, an enlarged right level II node was found later at the time of tonsillectomy. Biopsy and immunohistochemistry revealed classic histological findings of Kaposi's sarcoma. Immunohistochemistry stains for CD31, Factor VIII, and
vimentin were positive. Both
patients showed no recurrence after three years of follow up. HIV tests in both patients were negative and an immunodeficiency panel revealed normal levels of immunoglobulins, B-cells, T-cells, and NK cells.
A 2011 case report by Ozbudak3 et
al. also provided an example of classic tonsillar Kaposi’s sarcoma in an HIV-negative patient. The 68-year-old male from Turkey presented with sore throat and dysphagia along with cervical lymphadenopathy. No cutaneous lesions were present. Physical exam was significant for bilateral tonsillar masses. The masses were resected and further examination revealed hemorrhage and gray-white nodules. Histologic examination was diagnostic of Kaposi’s sarcoma with spindle cells, extravasated
red blood cells, and vascular slits. Tumor cells were positive for HHV-8, CD31, CD34
but negative for Factor VIII. Interestingly, microinvasive squamous cell carcinoma was detected along with a seven
mm Kaposi's sarcoma lesion in the lower third of the esophagus. After chemotherapy treatment, patient was disease free at two
and half years of follow-up.
Kaposi's sarcoma of the tonsils is extremely rare and only a
few cases have been described in the literature. Because of this, it can easily
be missed or misdiagnosed. Although Kaposi's sarcoma is strongly associated
with immunodeficiency, the possibility of Kaposi's sarcoma in HIV-negative
patients cannot be disregarded, especially in individuals of Mediterranean and
Eastern European descent. It is important to note that in the majority of cases
of tonsillar Kaposi's sarcoma described, no other cutaneous lesions were
present. Because of this, otolaryngologists should be aware that enlargement of
tonsils may be the initial signs of tonsillar Kaposi's sarcoma and any
suspicion of malignancy should warrant biopsy. Histologic identification
remains the only confirmatory diagnosis of Kaposi's sarcoma and will often show
characteristic spindle cells, vascular slits, and extravasated erythrocytes.
Staining for endothelial markers (CD31, CD34 and Factor VIII) is positive,
confirming a vascular origin for this neoplasm.
HHV-8 infection is widely considered the etiologic agent of
Kaposi's sarcoma.1 This virus has also been implicated in the progression
of multicentric Castleman's disease and primary
effusion lymphoma.9 Although its pathogenesis is not completely
understood, it has been known to stimulate various oncogenes, many of which are
involved with angiogenesis.10 Interestingly, HHV-8 has been reported
to replicate in the oropharynx and titers of the virus are highest in human
saliva when compared to plasma, semen, and other bodily secretions.11,12 Therefore,
although HHV-8 had initially been described to be transmitted sexually, new
evidence seems to suggest that oral-oral contact may provide an additional
route of transmission.
Based on the review of literature, the most common symptoms
associated with tonsillar Kaposi's sarcoma were dysphagia and cervical
lymphadenopathy. Other common features include sore throat and a mass in the
tonsillar fossa with associated hemorrhage or ulcerations. Current treatment
options include tonsillectomy, which may prove sufficient for isolated lesions.
Other treatment options including chemotherapy have also shown promise.3
We encourage physicians to be aware of primary
Kaposi's sarcoma of the tonsils when examining oral lesions or masses. If such
a clinical suspicion exists, the importance of histopathological examination
cannot be overstated and could be crucial in patient workup.
Acknowledgements
We would like to
thank all the staff members of the Department of Otolaryngology for their
support and encouragement.
Author Affiliations
1.Dr. Shivam Saxena, Jefferson Medical
College, Class of 2015, Department of Pathology, Anatomy and Cell Biology,
Jefferson University Hospital, 132S 10th St. Main Bldg. r285, Philadelphia, PA,
19107, 2.Dr. Joseph Curry, MD, Department of Otolaryngology Jefferson Univ. Hospital, 925 Chestnut St, 6th
floor, Philadelphia, PA, 19107, 3.Dr. Madalina Tuluc, MD, Department of Pathology, Anatomy and Cell
Biology, Jefferson University Hospital, 132S 10th St. Main Bldg. r285,
Philadelphia, PA, 19107.
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Corresponding Author
Dr. Shivam Saxena, BS,
Jefferson Medical College,
Class of 2015, Department of Pathology,
Anatomy and Cell Biology,
Jefferson University Hospital,
132S 10th St. Main Bldg. r285,
Philadelphia, PA, 19107.
Email: shivam.saxena@jefferson.edu
Source of Support:
Nil, Conflict of Interest: None Declared
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