Clinical features of 13 EMPs in children
| Patient . | Age (y)/gender . | Involved site . | Presenting symptoms . | Staging IPNHLSS . | Staging methods . | Urine/serum paraprotein (electrophoresis) . | Treatment . | Local relapse (time from ID) . | Follow-up, mo . | Outcome last FU . | Underlying Immuno-deficiency∗ . | Associated infection/autoimmune disorder† . |
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| 1 | 4/F | Tonsil | Snoring and frequent infections | I-EN | PET-CT, BMT, BMA, LP, and MRI | No‡/no | Resection at ID and relapse, w&w | Yes (2 and 3 years after ID) | 37 | Local relapse§ | No | No |
| 2 | 6/M | Palatal arch | Symptoms of adenoid hyperplasia and incidental finding of small tumor during adenectomy | I-EN | MRI, BMT, and bone scan | na/na | Resection, w&w | No | 12 | CR (CT) | No | No |
| 3 | 4/M | Tonsil (adenoid) | Progressing nasal breathing difficulties (3 weeks) | I-EN | PET-CT, BMT, and BMA | No/no | Resection, w&w | No | 24 | CR (PET-MRI) | No | No |
| 4 | 6/F | Eyelid | Tumor right upper median eyelid increasing over months | I-EN | MRI (whole body) and BMA | na/no | Incomplete resection, w&w | No | 12 | CR (MRI) | No | No |
| 5 | 3/M | Tonsil (adenoid) | Snoring, progressing enlargement of tonsils and adenoids, and cervical lymphadenopathy (5-6 months) | I-EN | MRI (whole body), BMA, and LP | na/na | Resection, w&w | No | 96 | CR (MRI) | No | No |
| 6 | 4/F | Tonsil (adenoid) | Upper airway obstruction | I-EN | PET-CT, BMT, BMA, and LP | No/no | Resection at ID and relapse, w&w | Yes (10 months and 3 years after ID) | 102 | CR (MRI), rebiopsy of initial site | No | No |
| 7 | 9/M | Larynx, vestibulum and supraglottis | Increasing dysphonia over 3-4 months; at diagnosis loss of voice | I-EN | PET-CT and BMA | No/no | Local irradiation (50.4 Gy), resection | No | 64 | CR (voice back to normal) | No | No |
| 8 | 6/M | Supraglottic tumor | Intermittent hoarseness for 3 years; progressing snoring and breathing difficulties for 6 months | I-EN | MRI, sonography, and laryngoscopy | na/no | Resection, w&w | No | 19 | CR (MRI, voice nearly normal) | No | Acute EBV (IgM+) infection |
| 9 | 18/F | Tonsil, bilateral | Clinical suspicion of EBV-associated tonsillitis | I-EN | CT, BMT, and FCM | No/no | Resection at ID and relapse, w&w | Yes (20 months after ID)|| | 24 | CR | No | Borderline EBV-IgM suspicion of EBV infection |
| 10 | 6/M | Adenoid | Reduced hearing and adenectomy | I-EN | BMT, FCM, and PET-MRI | No/no | Resection, w&w | No | 17 | CR | No | No |
| 11 | 15/F | Epipharynx, left conjunctiva | Adenoids and flat conjunctival lesion | II-EN | MRI and PET-CT | na/na | Resection, w&w | No | 243 | CR | No | No |
| 12 | 4/M | Epipharynx | Adenoid vegetations, chronic rhinitis, and recurrent otitis media | I-EN | MRI, bone scan¶, and BMA | No/no | Resection, w&w | No | 52 | CR | No | No |
| 13 | 4/M | Epipharynx | Adenoid vegetations, obstructive bronchitis, and breathing problems | I-EN | MRI, bone scan¶, and BMA | No/no | Resection, w&w | No | 85 | CR | No | No |
| Patient . | Age (y)/gender . | Involved site . | Presenting symptoms . | Staging IPNHLSS . | Staging methods . | Urine/serum paraprotein (electrophoresis) . | Treatment . | Local relapse (time from ID) . | Follow-up, mo . | Outcome last FU . | Underlying Immuno-deficiency∗ . | Associated infection/autoimmune disorder† . |
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| 1 | 4/F | Tonsil | Snoring and frequent infections | I-EN | PET-CT, BMT, BMA, LP, and MRI | No‡/no | Resection at ID and relapse, w&w | Yes (2 and 3 years after ID) | 37 | Local relapse§ | No | No |
| 2 | 6/M | Palatal arch | Symptoms of adenoid hyperplasia and incidental finding of small tumor during adenectomy | I-EN | MRI, BMT, and bone scan | na/na | Resection, w&w | No | 12 | CR (CT) | No | No |
| 3 | 4/M | Tonsil (adenoid) | Progressing nasal breathing difficulties (3 weeks) | I-EN | PET-CT, BMT, and BMA | No/no | Resection, w&w | No | 24 | CR (PET-MRI) | No | No |
| 4 | 6/F | Eyelid | Tumor right upper median eyelid increasing over months | I-EN | MRI (whole body) and BMA | na/no | Incomplete resection, w&w | No | 12 | CR (MRI) | No | No |
| 5 | 3/M | Tonsil (adenoid) | Snoring, progressing enlargement of tonsils and adenoids, and cervical lymphadenopathy (5-6 months) | I-EN | MRI (whole body), BMA, and LP | na/na | Resection, w&w | No | 96 | CR (MRI) | No | No |
| 6 | 4/F | Tonsil (adenoid) | Upper airway obstruction | I-EN | PET-CT, BMT, BMA, and LP | No/no | Resection at ID and relapse, w&w | Yes (10 months and 3 years after ID) | 102 | CR (MRI), rebiopsy of initial site | No | No |
| 7 | 9/M | Larynx, vestibulum and supraglottis | Increasing dysphonia over 3-4 months; at diagnosis loss of voice | I-EN | PET-CT and BMA | No/no | Local irradiation (50.4 Gy), resection | No | 64 | CR (voice back to normal) | No | No |
| 8 | 6/M | Supraglottic tumor | Intermittent hoarseness for 3 years; progressing snoring and breathing difficulties for 6 months | I-EN | MRI, sonography, and laryngoscopy | na/no | Resection, w&w | No | 19 | CR (MRI, voice nearly normal) | No | Acute EBV (IgM+) infection |
| 9 | 18/F | Tonsil, bilateral | Clinical suspicion of EBV-associated tonsillitis | I-EN | CT, BMT, and FCM | No/no | Resection at ID and relapse, w&w | Yes (20 months after ID)|| | 24 | CR | No | Borderline EBV-IgM suspicion of EBV infection |
| 10 | 6/M | Adenoid | Reduced hearing and adenectomy | I-EN | BMT, FCM, and PET-MRI | No/no | Resection, w&w | No | 17 | CR | No | No |
| 11 | 15/F | Epipharynx, left conjunctiva | Adenoids and flat conjunctival lesion | II-EN | MRI and PET-CT | na/na | Resection, w&w | No | 243 | CR | No | No |
| 12 | 4/M | Epipharynx | Adenoid vegetations, chronic rhinitis, and recurrent otitis media | I-EN | MRI, bone scan¶, and BMA | No/no | Resection, w&w | No | 52 | CR | No | No |
| 13 | 4/M | Epipharynx | Adenoid vegetations, obstructive bronchitis, and breathing problems | I-EN | MRI, bone scan¶, and BMA | No/no | Resection, w&w | No | 85 | CR | No | No |
BMA, bone marrow aspirate; BMT, bone marrow trephine biopsy; CR, complete remission; CT, computed tomography; F, female; FCM, flow cytometry; FU, follow up; ID, initial diagnosis; IPNHLSS, International Pediatric Non-Hodgkin Lymphoma Staging System; LP, lumbar puncture; M, male; MRI, magnetic resonance imaging; na, not available; nd, not done; PET, positron emission tomography; w&w, watch and wait.
No clinical evidence of an underlying immune defect or autoimmunity.
Associated infection specifically mentioned within the medical records and/or confirmed by serology.
Doubtful urine paraprotein measured once.
Child was lost to follow-up immediately after diagnosis of second local relapse.
The patient developed new adenoid-like proliferations 20 months after the initial diagnosis. Histopathology revealed a small area (10%) of the EMP and reactive hyperplasia in the remaining specimen; serum immune-fixation was negative at relapse.
Technetium (99Tc) bone scintigraphy.