Well-differentiated renal neuroendocrine tumors are rare tumors. As their biologic behavior is not fully known, there is a need to know more about these cases. We performed a retrospective chart review of all the cases diagnosed with renal neuroendocrine tumors from January 2016 to December 2020 (five years) in order to understand their clinical features, morphological characteristics and outcome. We included six cases with mean age of 46.2 years (4 males) in our study. All patients underwent radical nephrectomy. Histologically all showed tumor disposed in nests and trabeculae and majority of the tumors belonged to well-differentiated neuroendocrine tumor Grade 1 (WHO criteria of gastoroenteropancreatic neuroendocrine neoplasms). Lymph node metastasis was seen in two cases at the time of clinical presentation. All the tumors were diffusely positive for neuroendocrine tumor markers (synaptophysin, chromogranin, NSE, CD56). Follow-up data was available in all cases with an average follow-up of two years and neither has shown evidence of metastasis or relapse till last follow-up. Role of morphological patterns and immunohistochemical markers is highlighted with the importance of including Ki-67 index in grading them to better understand their outcome.

Goltz syndrome or focal dermal hypoplasia is a rare meso-ectodermal, X-linked dominant genetic disorder with female preponderance. Though the involvement of multiple systems is present, the hallmark of this syndrome is typical cutaneous features. Here, we report a unique case of Goltz syndrome in a 21-year-old male who presented to us with multiple growths on the palpebral conjunctiva of the right eye along with abnormal ice pick–type lesions on the skin. No significant family history was present. The affected eye also had iris and choroidal coloboma. Mild mental retardation and gingival hyperplasia were noted on systemic examination. Being a multisystem disorder, an ophthalmologist should be aware of its extraocular manifestations that can be fatal.

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