Department of Otolaryngology-Head and Neck Surgery, Jichi Medical University, School of Medicine, Tochigi, Japan Department of Otolaryngology, Ishibashi General Hospital, Tochigi, Japan.
Published: August 2015
AI Article Synopsis
Article Abstract
Background: Blue nevus is a benign dermal melanocyte tumor that mainly arises from the skin. We report an extremely rare case of blue nevus in a pediatric patient with extensive progression from the middle ear and inner ear to the nasopharynx through the Eustachian tube.
Case Report: A 2-year-old girl with blue tympanum was referred to our department. Computed tomography scans and magnetic resonance imaging were performed, followed by a tissue biopsy and histopathologic evaluations. Radiologic examinations revealed that the lesion had progressed beyond the middle ear into the inner ear and the nasopharynx through the Eustachian tube. Subsequent histopathologic examinations indicated dermal dendritic melanocytic proliferations, but no evidence of malignancy. Based on the clinical and histopathologic findings, we concluded that the lesion was consistent with blue nevus.
Discussion: Blue nevus is a relatively common skin lesion. However, no prior reports have described the extension of blue nevus from the auditory organ to the nasopharynx in a pediatric patient. Despite the benign nature of the lesion, the patient experienced profound hearing loss in the affected ear, which necessitates continued monitoring as the lesion may expand with patient growth.
Inferior vena cava aneurysms (IVCAs) are rare yet potentially lethal, especially if they are symptomatic or complicated. Among the IVCAs reported in the literature, only a few are associated with congenital vascular anomalies, including congenital IVC obstruction, tetralogy of Fallot, left-sided IVC, duplicated IVC, Ehlers-Danlos syndrome, blue rubber bleb nevus syndrome, and Klipper-Trenaunay syndrome. We present the case of an 8-cm symptomatic saccular IVCA in a patient with tetralogy of Fallot, treated successfully with surgical repair.
Background: Blue rubber blister nevus syndrome (BRBNS) is a congenital, rare disease characterized by venous malformations of the skin and internal organs, affecting all systems throughout the body. The pathogenesis is unknown. There is no consensus on the treatment of BRBNS.
Bean syndrome (BS) is a rare congenital angiomatosis affecting multiple organ systems. The exact etiology is still not well-known. About 200 cases with heterogeneous clinical presentations have been reported worldwide.
A 79-year-old man with fatigue and melena was diagnosed with severe iron-deficiency anemia. Capsule endoscopy revealed submucosal vascular lesions in the jejunum and active bleeding in the ileum, consistent with Blue Rubber Bleb Nevus Syndrome (BRBNS). Exploratory laparoscopy led to the resection of a 120 cm intestinal segment, confirming the diagnosis.
Melanoma arising in association with a blue nevus (BN) is rare but has molecular similarities to uveal melanoma (UM), including GNAQ/11 mutations. Tebentafusp was recently approved for UM based on improved overall survival in a phase 3 study. We hypothesized that tebentafusp may be active in BN-associated melanoma.
