Epistaxis and cervical lymphadenopathy in a 6-year-old child - Ominous symptom complex hinting underlying malignancy

Dear Editor,

Cervical lymphadenopathy in children is a routinely encountered condition in paediatric practice. Some common causes include reactive lymphadenitis, infections such as tuberculosis, lymphoproliferative disorders and rarely metastasis.^[1] For early diagnosis of neoplastic causes, cervical lymphadenopathy in suspected cases warrants fine-needle aspiration cytology (FNAC). Although lymph node FNAC is a simple and rapid procedure, it should not be regarded as the standard initial diagnostic test. Lymph node biopsy remains the gold standard for definitive histological confirmation.

Bleeding from the nostrils (Epistaxis) in a child can be due to local trauma, deviated nasal septum, systemic hypertension, bleeding disorder and thrombocytopenia. Neoplasms that present with epistaxis in children are acute leukaemia with thrombocytopenia, lymphoma, juvenile nasopharyngeal angiofibroma and nasopharyngeal carcinoma (NPC). Recurrent epistaxis in children needs a thorough diagnostic workup to rule out an underlying neoplastic cause.^[2]

We report one such case of epistaxis with cervical lymphadenopathy, which turned out to be due to an underlying malignancy.

A 6-year-old male presented with three episodes of spontaneous, profuse epistaxis from the right nostril over 1 week. There was no history of trauma, fever, nasal obstruction, or other mucocutaneous bleeding. On examination, the child was alert and active. Anterior rhinoscopy was unremarkable, but posterior rhinoscopy revealed a friable nasopharyngeal mass with postnasal bleeding. Multiple tender, mobile cervical lymph nodes were palpable, the largest being 1 × 1 cm in the left posterior triangle [Figure 1a].

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Figure 1:

(a) Left posterior cervical lymphadenopathy in a male child, (b) Fine-needle aspiration cytology of the left cervical lymph node showed loosely cohesive sheets of undifferentiated tumours cells with round nuclei, finely dispersed chromatin and prominent nucleoli with surrounding lymphocytes (haematoxylin and eosin [H&E] stain, ×100) (c) Magnetic resonance imaging of the head and neck showing soft-tissue density mass (black arrow) arising from nasopharynx. (d) Biopsy of nasopharyngeal mass showed syncytial sheets of undifferentiated cells with round nuclei, vesicular chromatin, prominent nucleoli and lymphoplasmacytic infiltrates (H&E stain, ×400).

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FNAC from the cervical node showed loosely arranged sheets of undifferentiated cells with eosinophilic cytoplasm, vesicular chromatin and prominent nucleoli, surrounded by lymphocytes and plasma cells [Figure 1b]. Magnetic resonance imaging revealed a 4 × 3 cm infiltrative mass involving the left skull base and nasopharynx, with sphenoid bone destruction and extension to the petrous temporal bone and clivus [Figure 1c]. Transnasal endoscopic biopsy from the mass showed syncytial sheets of undifferentiated cells with prominent lymphoplasmacytic infiltrate [Figure 1d]. Cervical node biopsy confirmed metastasis from undifferentiated NPC. Computed tomography chest and abdomen ruled out distant metastasis. Serum immunoglobulin A against Epstein–Barr virus (EBV) was elevated. Based on clinical, radiological, cytological and histopathological findings, a diagnosis of undifferentiated NPC, stage III (T3, N1, M0), was made. The child was referred for oncology management.

In a child presenting with epistaxis and cervical lymphadenopathy, a thorough examination of the Ear, Nose, and Throat (ENT) is essential to detect underlying malignancy and guide biopsy.^[1] NPC is a rare pediatric epithelial malignancy (<1% of childhood cancers), with peak incidence in adolescence.^[3] Below 10 years, NPC often presents atypically with cervical lymphadenopathy or epistaxis. Due to its deep location, diagnosis is frequently delayed. NPC is strongly associated with EBV, which contributes to malignant transformation by inactivating tumor suppressor genes.

Early diagnosis allows treatment during localised stages, improving outcomes. Stage I–II NPC has >90% 5-year survival, compared to <70% in stage III–IV disease.^[4] It also permits less intensive treatment, minimising long-term complications such as xerostomia, ototoxicity or growth disturbances. In our case, early ENT evaluation and prompt biopsy enabled timely diagnosis at stage III.

We report a rare case of undifferentiated NPC in a 6-year-old, presenting with epistaxis and cervical lymphadenopathy. This highlights the need for high clinical suspicion, detailed ENT examination and early tissue diagnosis in any child with persistent, unexplained cervical lymphadenopathy or nasal symptoms, to rule out malignancy and initiate timely treatment.