Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0242429 (sore throat)
 2,760 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Kaposiform hemangioendothelioma (KHE) is an uncommon vascular tumor of intermediate malignant potential, usually occurs in the extremities and retroperitoneum of infants and is characterized by its association with lymphangiomatosis and Kasabach-Merritt phenomenenon (KMP) in certain cases. It has rarely been observed in the head and neck region and at times, can present without KMP. Herein, we present an extremely uncommon case of KHE occurring in tonsil of a child, associated with a neck swelling, but unassociated with KMP. A 2-year-old male child referred to us with history of sore throat, dyspnoea and right-sided neck swelling off and on, since birth, was clinicoradiologically diagnosed with recurrent tonsillitis, including right sided peritonsillar abscess, for which he underwent right-sided tonsillectomy, elsewhere. Histopathological sections from the excised tonsillar mass were reviewed and showed a tumor composed of irregular, infiltrating lobules of spindle cells arranged in kaposiform architecture with slit-like, crescentic vessels. The cells displayed focal lumen formation containing red blood cells (RBCs), along with platelet thrombi and eosinophilic hyaline bodies. In addition, there were discrete foci of several dilated lymphatic vessels containing lymph and lymphocytes. On immunohistochemistry (IHC), spindle cells were diffusely positive for CD34, focally for CD31 and smooth muscle actin (SMA), the latter marker was mostly expressed around the blood vessels. Immunostaining for HHV8 was negative and Ki-67 (proliferation marker) displayed focal positivity. Diagnosis of KHE was made. Platelet count was towards lower side of range. Postoperative imaging showed discrete, multiple fluid containing lesions in the right neck that were high on T2-weighed sequences, on magnetic resonance imaging (MRI) and ipsilateral intraoral mucosal growth. Fine needle aspiration cytology (FNAC) smears from neck swelling showed blood, fluid and lymphocytes. Possibility of a coexisting lymphangioma was considered. The patient was offered sclerotherapy and is on follow-up. This case forms the second documented case of KHE at this site, along with its unique association with neck lymphangioma. KHE has distinct histopathological features and can be sorted out from its other differentials like juvenile hemangioma and Kaposi's sarcoma. IHC stains are useful in substantiating a definite diagnosis.
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PMID:Kaposiform hemangioendothelioma in tonsil of a child associated with cervical lymphangioma: a rare case report. 2160 41

Tonsillar involvement in Kaposi sarcoma is extremely rare, as only a few such cases have been reported; all but 1 of these previously reported cases occurred in patients with human immunodeficiency virus (HIV) infection. We describe what to the best of our knowledge is the first reported case of concurrent bilateral tonsillar and esophageal Kaposi sarcoma in an HIV-negative patient. A 68-year-old man presented with sore throat and dysphagia. Clinical examination revealed the presence of bilateral and asymmetrical tonsillar masses, as well as generalized lymphadenopathy in the cervical chain. The masses were resected, and findings on histopathologic analysis were consistent with Kaposi sarcoma. In addition, human herpesvirus 8 was demonstrated on a tonsil specimen by polymerase chain reaction, and microinvasive squamous cell carcinoma was also detected. Later, another Kaposi sarcoma lesion was detected in the lower third of the esophagus. We recommend that clinicians not discount the possibility of oral classic Kaposi sarcoma in the workup of an immunocompetent patient with oral vascular lesions.
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PMID:Bilateral tonsillar and esophageal Kaposi sarcoma in an HIV-negative patient. 2179 87

Basic knowledge concerning the human immunodeficiency virus (HIV) and acquired immunodeficiency syndrome (AIDS) is useful for ENT physicians. Although HIV patients are usually stably asymptomatic nowadays due to modern therapy, HIV often manifests in ENT symptoms, such as neck lumps, sore throat, difficulty swallowing and dysgeusia. After infection, an initial increase in viral load can cause, among other symptoms, oral ulcers and pharyngitis. Once the immune system is compromised by the attack on CD4 lymphocyte cells, HIV-related diseases can occur: oral mycoses (particularly candidosis) and viral infections (including warts), aphthous ulcers, gingivitis, salivary gland diseases and malignancies (e. g. intraoral Kaposi's sarcoma). Neck lymphadenopathy is frequent. Markers of disease severity are the clinical symptoms, viral load and CD4 helper cell count. HIV treatment (antiretroviral therapy, ART) is a combination of at least three antiviral drugs.
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PMID:[A practically orientated clinical HIV update for ENT physicians]. 2535 97