Hemangiomas are benign, slow-growing tumors composed of sinusoidal blood vessels. Skeletal hemangiomas are uncommon and are mostly vertebral, followed by cranial in location. Cranial hemangiomas are very rarely encountered in children. Authors report a 12-year girl who presented with a painless enlarging mass over the parietal scalp for 3 months. Imaging revealed a left parietal intraosseous lytic mass with a sunburst appearance. Enbloc removal and cranioplasty was performed, and histopathology was suggestive of hemangioma. We reviewed the literature on pediatric cranial intraosseous hemangiomas (PCIH) (age ≤18 years) by searching online database. Including ours, a total of 24 cases were analyzed. Mean age was 10.2 years (range 4 months-17 years). Eight were in the first decade and 16 were in the second decade. Male:female ratio was 12:12 (1:1). A painless palpable mass was the commonest presenting feature. Parietal and frontal bones were most commonly involved. Intracranial extension was noted in cases. Mean size of the lesion was 5 cm (range 1-12 cm). Twenty-two underwent primary surgical removal while two had additional pre-operative embolization. Surgical procedures were craniectomy alone (n-3), craniectomy + cranioplasty (n-6), tumor excision + remodeling (n-3), and tumor debulking (n-2). Histopathology was cavernous type in majority of cases. Mean follow-up duration was 11.8 months (range 2-38 months). There were no recurrences. One patient died due to systemic infection. Ours is the first review exclusively on PCIH. Although rare, they need to be considered in the differential diagnosis of lytic skull lesions in children. Enbloc removal with cranioplasty is the preferred treatment in vault hemangiomas, while embolization followed by debulking would suffice in large cranial base lesions.
Livedoid vasculopathy with mononeuritis multiplex is a rare association. We are presenting a case of an unusual association of livedoid vasculopathy with mononeuritis multiplex, who developed fulminant hepatic failure which was secondary to Herpes simplex virus (HSV) hepatitis, while she was on treatment with immunosuppressants. Her skin biopsy and immunofluorescence studies showed the features of vasculitis. A biopsy from the sural nerve showed the features of chronic vasculitis.
A moderate to strong inter-membranous E-Cadherin expression on immunohistochemistry was seen in all the cases of IDC, while only 1 case of ILC showed a moderate E-Cadherin expression. Hence, the E-cadherin expression can be reliably used as a marker to differentiate IDC and ILC. However, an aberrant cytoplasmic expression of E-Cadherin may be seen in some cases of ILC, which should be interpreted with caution.
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