Superficial Parotidectomy for Juvenile Recurrent Parotitis.

OBJECTIVES: To investigate long-term outcomes, imaging, and pathologic findings in pediatric patients who underwent superficial parotidectomy for recalcitrant juvenile recurrent parotitis (JRP). METHODS: Records for 20 children (23 parotidectomies; 9 females, 11 males; age at surgery of 8.6 ± 3.7 years) collected over a 10-year period (2012-2021) were reviewed. Parents were contacted via telephone to obtain extended follow-up. A simplified scoring system was used to assess imaging findings and an additional pathologic review was conducted to further clarify the underlying disease process. RESULTS: All but one patient experienced resolution of their recurrent symptoms after superficial parotidectomy. Three of the patients studied required surgery on the contralateral side, and this could be predicted based on their imaging at the time of the initial surgery. Pathologic findings included ductal fibrosis, metaplasia, and dilatation as well as parenchymal atrophy and fatty deposition. There were no major surgical complications, however, the incidence of Frey's syndrome in this sample was 43.5% of surgical sites. CONCLUSION: For patients with frequent recalcitrant symptoms or significant quality of life impairment related to JRP, superficial parotidectomy represents a potential treatment option with the noted reduction in symptom burden following surgery. Further longitudinal studies are needed. LEVEL OF EVIDENCE: 4 Laryngoscope, 133:1495-1500, 2023.

Neuroblastoma Arising in an Immature Teratoma of the Ovary in a 13-Year-Old.

Somatic malignancies arising in mature teratomas are exceedingly rare entities and even more so are those arising in immature teratomas. Here, we present a unique case of a 13-year-old who initially underwent ovarian sparing cystectomy for a 7.7 cm left ovarian mass with a pre-operative diagnosis of mature cystic teratoma. Histologically, all 3 germ cell layers were present and immature neuroepithelial tubules were also identified. Subsequent sections revealed a nodular lesion composed of neuropil, neuroblasts with a spectrum of maturation, and Schwannian-type stroma. The neuroblasts were diffusely positive for PHOX2B. Neuroblastoma arising in an immature teratoma has only been described in the literature once previously in an adult patient.

Tufted Angioma of the Index Finger Middle Phalanx.

A tufted angioma is a benign vascular tumor of the skin and subcutaneous tissue that classically presents as a violaceous nodule on the trunk or extremities in early childhood. Tufted angiomas of the finger are uncommon, and intraosseous involvement of these tumors is exceedingly rare. When present in the bone, these lesions may be difficult to distinguish from the more common pediatric condition of osteomyelitis or osteoid osteoma. We present the clinical, radiographic, and histopathologic findings for a unique case of a tufted angioma with intraosseous involvement in the middle phalanx treated by surgical excision and curettage with preservation of function.

Undifferentiated and Unresectable Sarcoma With NTRK3-Fusion in a Pediatric Patient Treated With Larotrectinib and Proton Beam Radiotherapy.

A 6-year-old female presenting with an abdominal mass was found to have an unresectable undifferentiated sarcoma. The tumor did not respond to multiagent chemotherapy. However, molecular testing identified an NTRK3-fusion, and treatment was changed to larotrectinib monotherapy. Following 6 months of therapy, the patient achieved a very good partial response with 96% reduction in tumor size. She underwent proton beam radiation therapy with continued larotrectinib therapy and achieved a complete response. This case report shows that an NTRK fusion positive undifferentiated sarcoma can be safely treated with larotrectinib and radiation therapy and highlights the importance of early molecular testing.

Intravascular large B-cell lymphoma presenting with altered mental status: A case report.

BACKGROUND: Intravascular large B-cell lymphoma (IVLBCL) is a rare and aggressive subtype of non-Hodgkin lymphoma with a varied presentation and no pathognomonic findings. Early diagnosis is critical to altering the disease course as early treatment with chemoimmunotherapy is required to prevent a rapidly fatal outcome. Strategies including improved awareness of this clinical entity through publication of cases with unique presentations are essential to prompt consideration of IVLBCL early in the disease workup. Here, we present a case of IVLBCL presenting with altered mental status and systemic organ dysfunction. CASE SUMMARY: A 61-year-old male patient presented with flu-like symptoms and a high fever. He experienced rapid clinical deterioration with liver, kidney failure, and shock despite rapid antibiotic administration and supportive care. A broad infectious workup was negative. Intracranial imaging revealed nonspecific changes to the corpus callosum suspicious for vasculitis. Renal biopsy was non-diagnostic. After further progression of his symptoms, the family elected to withdraw care and the patient died shortly thereafter. Post-mortem analysis revealed clear multi-organ involvement by IVLBCL, prompting re-examination of the ante-mortem renal biopsy that also identified IVLBCL involvement. CONCLUSION: IVLBCL is a rare disease. Communication with specialties and early biopsy is critical to establishing the diagnosis and initiating therapy.