International Journal of Pediatric Otorhinolaryngology 78 (2014) 885–887
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Leukemoid reaction associated with pediatric nasopharyngeal carcinoma: An unusual presentation Asiye Burcu Kus¸ a, Poyraz S¸ahin b, Aysun Hatice Ug˘uz c, Atila Tanyeli d, Serhan Ku¨peli d,* a
C¸ukurova University, Faculty of Medicine, Department of Pediatrics, Turkey C¸ukurova University, Faculty of Medicine, Department of Otorhinolaryngology, Turkey c C¸ukurova University, Faculty of Medicine, Department of Pathology, Turkey d C¸ukurova University, Faculty of Medicine, Department of Pediatric Oncology/Pediatric BMT Units, Turkey b
A R T I C L E I N F O
A B S T R A C T
Article history: Received 17 December 2013 Received in revised form 6 February 2014 Accepted 7 February 2014 Available online 20 February 2014
Nasopharyngeal carcinoma is a tumor originating from the surface epithelial cells of nasopharynx. It is rare in children and adolescents. Most common physical ﬁnding is a neck mass. Most children with nasopharyngeal carcinoma present with advanced stage disease. The presentation with hematological abnormalities in patients without systemic metastasis is extremely rare. We reported a 14-year-old boy presenting with a mass at the right side of the pharynx and leukemoid reaction. To our knowledge, this is the ﬁrst report of leukemoid reaction associated with pediatric nasopharyngeal carcinoma in English literature. ß 2014 Elsevier Ireland Ltd. All rights reserved.
Keywords: Leukemoid reaction Nasopharyngeal carcinoma Childhood
1. Introduction Nasopharyngeal carcinoma (NPC) is a rare tumor accounting for approximately 1% of all childhood tumors. It also accounts for 20– 50% of all primary nasopharyngeal tumors in this age group . Undifferentiated variant is the most common form. The malignancy shows a bimodal age distribution with an early maximum age between 10 and 20 years and a second of 40 and 60 years. Current data suggests that there are some etiologic factors: viral (especially EBV), genetic and environmental but the etiology of NPC remains undetermined . The clinical presentation usually consists of nasal obstruction, change in hearing, cranial nerve palsy, serous otitis . Most common physical ﬁnding is a neck mass in 80% of patients. Leukemoid reaction is described as an increase in white blood cell count similar to what occurs in people with leukemia. Tumor-related leukemoid reaction is a kind of neoplastic syndrome, not due to an infection or other disease. Our patient presented with leukemoid reaction which has not yet reported in pediatric patients with NPC. 2. Case report A 14-year-old boy presented with headache that was resistant to analgesics for three weeks. He was diagnosed as sinusitis at
* Corresponding author. Tel.: +90 5357634549. E-mail address: [email protected]
(S. Ku¨peli). http://dx.doi.org/10.1016/j.ijporl.2014.02.016 0165-5876/ß 2014 Elsevier Ireland Ltd. All rights reserved.
another center and was given oral antibiotic treatment for sinusitis. Following headache he also complained of sore throat and a mass lesion at right side of the neck. MRI showed a mass lesion originating from right side of the nasopharynx. The patient was referred to otorhinolaryngology department of our hospital. At the time of presentation to otorhinolaryngology department, the patient had headache, dysphagia, dyspnea, hoarseness and cloudy vision at right eye. There was no history of fever, trauma, vertigo, facial paralysis, hearing loss, epistaxis and exposure to patients with tuberculosis. The patient had unremarkable family history. Physical examination revealed an ulcerating vegetative lesion about 2.0 cm 2.0 cm at right half of the pharynx. There were multiple bilateral servical lymph nodes prominently at right side, without palpable supraclavicular lymph nodes. At opthalmologic examination; right pupil was minimally mydriatic and direct/ indirect light reﬂexes were slightly deﬁcient. Neuromuscular system examination was normal. Maxillofacial MRI revealed a mass lesion measured about 6.0 cm 4.7 cm 4.0 cm originating from right half of the nasopharynx showing invasion to pterygoid muscles of lateral site; cavernous sinus at superior and meningeal area of anterior to right temporal lobe. The border of lesion was not uniform (Fig. 1). Complete blood count of patient showed WBC: 91.500/mm3, Hct: 29.8%, Plt: 602.000/mm3. There were 84% PMNL, 12% lymphocyte, 8% monocyte, no blast and atypical cells at peripheral blood smear. Following white blood cell counts were 61.000/mm3 and 67.300/ mm3 at 2nd and 3rd days. Acute phase reactants were found as
A.B. Kus¸ et al. / International Journal of Pediatric Otorhinolaryngology 78 (2014) 885–887
Fig. 1. Maxillofacial MR imaging.
erytrocyte sedimentation rate: 28 mm/h, procalcitonin: 0.1 ng/ml and C-reactive protein: 14.9 mg/dl. Uric acid levels, liver and kidney function tests were normal. An antibiotherapy consisting of cefaperasone and clindamycin was started with a suspicion of deep neck infection. An incisional lymph node biopsy was taken by otorhinolaryngologists with a suspicion of leukemoid reaction due to deep neck infection or abscess. Serologic markers for EBV infection showed past infection. No microorganisms were cultured in microbiological tests of a piece of the biopsy specimen. Histopathological examination revealed synctial appearing cells with vesicular nuclei, distinctive nucleoli and eosinophilic cytoplasm and some intermingled lymphocytes (Fig. 2a). Immunohistochemically pan-cytokeratin, cytokeratin-7 (CK7) and cytokeratin-20 (CK20) were performed to the tumor tissue and keratin was detected as positive (Fig. 2b) but the other two markers were negative. The biopsy was reported as undifferentiated carcinoma and a note was attached to the report that nasopharynx could be primary tumor site. Thoracoabdominal CT and bone scintigraphy of the patient showed no metastasis. Bone marrow biopsy revealed