ABSTRACT
Background: Neurenteric cysts are uncommon, benign endoderm-derived lesions that result from aberrant embryologic development of the notochord. They are typically located in the intradural extramedullary spinal cord and rarely located intracranially. Contrary to spinal-located cysts, intracranial cysts are rarer in the pediatric population. Clinically, they may present with symptoms of mass effect, or they can be incidentally discovered. Case Description: A 10-year-old healthy female child presented with recurrent headaches. The physical and neurological examination was unremarkable. Brain magnetic resonance imaging (MRI) showed a well-demarcated lesion anterior to the pontomedullary junction with striking T1 and T2/T2 fluid-attenuated inversion recovery high-signal intensity and a small rounded nodule within of low signal on T1, T2, and T2*. On initial conservative strategy with serial brain MRI, there was a progressive enlargement of the lesion with significant mass effect on the brainstem. The patient underwent a right retrosigmoid craniotomy, and the cyst wall was fenestrated and drained. Part of the cyst wall and the solid nodule were adherent to the brainstem and basilar artery and were not removed. The histologic findings were consistent with the diagnosis of a benign endodermal cyst. The postoperative period was uneventful. Conclusion: We report a successful surgical treatment of this rare congenital cyst located in the ventral brainstem. We present pre-and post-operative imaging findings, intraoperative microscopic images of the procedure, and a brief review of relevant clinical literature on the topic.
ABSTRACT
Sebaceous carcinoma of the vulva is a rare malignancy of the sebaceous glands, with potentially aggressive behaviour, that is usually found in the peri-ocular area. Nonetheless, there are sebaceous glands in the vulva and this diagnosis is especially rare, with only ten cases described in the literature. We report a case of 78-year-old female patient who presented with vulvar pruritus, previously treated with topical steroid and antifungal treatments, without improvement. The vulvar examination showed a visible yellow papule, 12 x 10 mm on the right major labia, which was biopsied and the microscopic examination revealed an invasive sebaceous carcinoma of the vulva, with an in situ component. We performed an uneventful excisional biopsy, followed by a subsequent margin widening. Three months after the diagnosis, she presented with the first recurrence. Two and half years after the diagnosis, she recurred with a larger lesion (13 mm) in the upper half of small right lip, more than 10 mm away from the midline. In a multidisciplinary meeting it was decided that the patient should undergo partial right vulvectomy with homolateral inguino-femoral sentinel node biopsy (one negative node). There was no evidence of recurrence one-year post-surgery.
O carcinoma sebáceo da vulva é uma neoplasia maligna rara das glândulas sebáceas com comportamento potencialmente agressivo, mais comummente encontrada na área periocular. Este diagnóstico é particularmente raro nas glândulas sebáceas da vulva, com apenas dez casos previamente descritos na literatura. Relatamos o caso de uma paciente de 78 anos que se apresentou com prurido vulvar, anteriormente tratado com corticóides tópicos e antifúngicos, sem melhoria evidente. Ao exame objetivo apresentava uma pápula amarela visível, com cerca de 12 x 10 mm no grande lábio direito, que foi biopsada. O exame microscópico revelou um carcinoma sebáceo invasivo da vulva, com componente in situ. Posteriormente, foi realizada uma biópsia excisional, com alargamento de margens, sem intercorrências. Três meses após o diagnóstico, surgiu a primeira recidiva, pelo que foi realizada uma nova biópsia excisional. Dois anos e meio após o diagnóstico, a doente apresentava uma lesão com 13 mm, na metade superior do pequeno lábio direito, mais de 10 mm afastada da linha mediana. Em reunião multidisciplinar decidiu-se que deveria ser submetida a uma vulvectomia parcial direita, com pesquisa de gânglio sentinela inguino-femoral homolateral (um gânglio negativo). Um ano após a cirurgia, a doente encontra-se sem evidência de recorrência.
