Tonsillar Kaposi's Sarcoma: Case Report and Review of Literature

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.

 

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, 6^th 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.

 

References

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3.     Ozbudak I, Guney K, Mutlu D, Gelen T, Ozbilim G. Bilateral tonsillar and esophageal kaposi sarcoma in an HIV-negative patient. Ear Nose Throat J 2011;90(7):E23-E26.

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7.     Jones R, Orchard GZ,B., Jones E. Immunostaining for CD31 and CD34 in kaposi sarcoma. J Clin Pathol 1995;48(11):1011-6.

8.     Sikora A, Shnayder Y, Yee H, De Lacure M. Oropharyngeal kaposi sarcoma in related persons negative for human immunodeficiency virus. Ann Otol Rhinol Laryngol 2008;117(3):172-6.

9.     Chagas C, Endo L, Sakano E, Pinto G, Brousset P, Vasallo J. Detection of herpesvirus type 8 (HHV8) in children's tonsils and adenoids by immunohistochemistry and in situ hybridization. Int J Pediatr Otorhinolaryngol 2006;70(1):65-72.

10.  Bais C, Santomasso B, Coso O, et al. G-protein-coupled receptor of kaposi's sarcoma-associated herpesvirus is a viral oncogene and angiogenesis activator. Nature 1998;391(6662):86-9.

11.  Myoung J, Ganem D. Active lytic infection of human primary tonsillar B cells by KSHV and its non-cytolytic control by activated CD4+ T cells. J Clin Invest 2011;121(3):1130-40.

12.  Pauk J, Huang M, Brodie S, et al. Mucosal shedding of human herpesvirus 8 in men. N Engl J Med 2000;343(19):1369-77.

 

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