Cardiac Tumors and the Nevoid Basal Cell Carcinoma Syndrome

PEDIATRICS ◽  
1991 ◽  
Vol 87 (5) ◽  
pp. 725-728
Author(s):  
JOHN L. COTTON ◽  
RAE-ELLEN W. KAVEY ◽  
CATHERINE E. PALMIER ◽  
WALTER W. TUNNESSEN

Nevoid basal cell carcinoma syndrome is a multisystem disease with a wide range of initial symptoms that can be seen at any age. The most characteristic features are vertebral or rib anomalies, intracranial falx calcification, multiple basal cell carcinomas, odontogenic keratocysts of the jaw, and palmar and/or plantar pits. Pediatricians need to be aware that if any one of these major anomalies is seen, this diagnosis should be considered. There now appears to be an established association between cardiac tumors and nevoid basal cell carcinoma syndrome. Primary cardiac tumors have been associated with cerebral tuberous sclerosis and neurofibromatosis,20,21 and evaluation of cardiac status is recommended when these genetically determined syndromes are diagnosed. This communication should serve to alert pediatricians to the need for complete cardiac evaluation and genetic counseling when a diagnosis of nevoid basal cell carcinoma is made.

2015 ◽  
Vol 8 (3) ◽  
pp. 536-539
Author(s):  
Ellen Toyonaga ◽  
Hiroo Hata ◽  
Chihiro Nakayama ◽  
Erina Homma ◽  
Toshiyuki Miyashita ◽  
...  

Nevoid basal cell carcinoma syndrome (NBCCS), also known as Gorlin syndrome, is a rare hereditary condition characterized by a wide range of developmental abnormalities and a predisposition to neoplasms. The syndrome consists of early-onset and/or multiple BCC. Herein we report a rare NBCCS case in which the first BCC onset occurred in the groin area. To the best of our knowledge, there have been no reports of first-onset BCC in the groin area in an NBCCS patient of any race.


2021 ◽  
Vol 11 (1) ◽  
pp. 1790-1802
Author(s):  
Reshmi Shrestha ◽  
Gita Sayami

Background: Eyelid pathologies are the most common surgical specimens encountered among all of the ophthalmic lesions and constitute a wide range of diseases by their unique histologic features. This study aims to find out the histopathological spectrum of eyelid lesions, their demographic distribution, and preferential location prevalent in our community.Materials and Methods: This is an observational study in which we retrospectively evaluated the data of 692 patients retrieved from the histopathology department of National Reference Laboratory, Kathmandu, from May 2016 to April 2019.Results: A total of 701 histologic diagnoses comprised of benign, precursor, and malignant lesions and accounted for 86.6%, 2.6%, and 10.8% respectively with preponderance in females. The common benign lesions included melanocytic nevus (17.7%), epidermal cyst (11%), hemangioma (8.9%), dermoid cyst (8.2%), chalazion (6.7%), and squamous papilloma (6.4%). Tumour of epidermal origin was the most common neoplastic lesion accounting for 31.2%. Basal cell carcinoma (50%) followed by sebaceous carcinoma (27.6%) and squamous cell carcinoma (14.5%) constituted the majority of malignant lesions prevalent above the age of 60 years with the preferential site of the upper eyelid for basal cell carcinoma and squamous cell carcinoma; and lower eyelid for sebaceous carcinoma.Conclusions: Benign eyelid lesions are more prevalent than malignant ones with overall female  preponderance. Epidermal tumours are common among neoplasms. A malignant tumour, a disease of  an elderly individual, is predominated by basal cell carcinoma followed by sebaceous carcinoma, an aggressive tumour with a high recurrence rate in our population.


Neurosurgery ◽  
1982 ◽  
Vol 11 (1_pt_1) ◽  
pp. 52-56 ◽  
Author(s):  
Mahmoud G. Naguib ◽  
Joo Ho Sung ◽  
Donald L. Erickson ◽  
Lawrence H. A. Gold ◽  
Edward L. Seljeskog

Abstract The characteristic features of central nervous system involvement in the nevoid basal cell carcinoma syndrome are reviewed. Emphasis is given to the frequent association of medulloblastoma with the syndrome.


2015 ◽  
Vol 33 (34) ◽  
pp. 4058-4065 ◽  
Author(s):  
Elizabeth K. Cahoon ◽  
Cari M. Kitahara ◽  
Estelle Ntowe ◽  
Emily M. Bowen ◽  
Michele M. Doody ◽  
...  

Purpose UV radiation exposure is the primary risk factor for basal cell carcinoma (BCC), the most common human malignancy. Although the photosensitizing properties of estrogens have been recognized for decades, few studies have examined the relationship between reproductive factors or exogenous estrogen use and BCC. Methods Using data from the US Radiologic Technologists Study, a large, nationwide, prospective cohort, we assessed the relationship between reproductive factors, exogenous estrogen use, and first primary BCC while accounting for sun exposure, personal sun sensitivity, and lifestyle factors for geographically dispersed women exposed to a wide range of ambient UV radiation. Results Elevated risk of BCC was associated with late age at natural menopause (hazard ratio [HR] for ≥ 55 years v 50 to 54 years, 1.50; 95% CI, 1.04 to 2.17) and any use of menopausal hormone therapy (MHT; HR, 1.16; 95% CI, 1.03 to 1.30; P for trend for duration = .001). BCC risk was most increased among women reporting natural menopause who used MHT for 10 or more years versus women who never used MHT (HR, 1.97; 95% CI, 1.35 to 2.87). Risk of BCC was not associated with age at menarche, parity, age at first birth, infertility, use of diethylstilbestrol by participant's mother, age at hysterectomy, or use of oral contraceptives. Conclusion These analyses confirm a previous finding of increased risk of BCC associated with MHT. Novel findings of increased BCC risk associated with MHT in women experiencing natural menopause and for late age at natural menopause warrant further investigation. Users of MHT may constitute an additional high-risk group in need of more frequent skin cancer screening.


Author(s):  
Victoria L. Wade ◽  
Winslow G. Sheldon ◽  
James W. Townsend ◽  
William Allaben

Sebaceous gland tumors and other tumors exhibiting sebaceous differentiation have been described in humans (1,2,3). Tumors of the sebaceous gland can be induced in rats and mice following topical application of carcinogens (4), but spontaneous mixed tumors of basal cell origin rarely occur in mice.


2000 ◽  
Vol 39 (5) ◽  
pp. 397-398 ◽  
Author(s):  
Hyoung-Joo Kim ◽  
Youn-Soo Kim ◽  
Ki-Beom Suhr ◽  
Tae-Young Yoon ◽  
Jeung-Hoon Lee ◽  
...  

1978 ◽  
Vol 114 (12) ◽  
pp. 1845-1845 ◽  
Author(s):  
G. P. Lupton

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