scholarly journals Sonidegib after vismodegib discontinuation in a patient with Gorlin‐Goltz Syndrome and multiple basal cell carcinomas

2021 ◽  
Author(s):  
Alfredo Piccerillo ◽  
Alessandro Di Stefani ◽  
Alessio Costantini ◽  
Ketty Peris
Keyword(s):  
Basal Cell ◽  
Goltz Syndrome ◽  
Basal Cell Carcinomas ◽  
Multiple Basal Cell Carcinomas

Multiple Basal Cell Carcinomas in a 38-Year-Old Woman with Goltz Syndrome

Dermatology ◽  
2012 ◽  
Vol 224 (2) ◽  
pp. 97-100
Author(s):  
Annalisa Patrizi ◽  
Michela Tabanelli ◽  
Karl-Heinz Grzeschik ◽  
Cosimo Misciali ◽  
Iria Neri ◽  
...  
Keyword(s):  
Basal Cell ◽  
Goltz Syndrome ◽  
Basal Cell Carcinomas ◽  
Multiple Basal Cell Carcinomas

Gorlin-Goltz Syndrome and Neoplasms: A Case Study

2010 ◽  
Vol 35 (2) ◽  
pp. 203-206 ◽  
Author(s):  
Nilza Lopes ◽  
Eliana Caran ◽  
Maria Lucia Lee ◽  
Nasjla Saba Silva ◽  
André Caroli Rocha ◽  
...  
Keyword(s):  
Basal Cell ◽  
Facial Dysmorphism ◽  
Autosomal Dominant Disorder ◽  
Craniospinal Radiation ◽  
Goltz Syndrome ◽  
Basal Cell Carcinomas ◽  
Vertebral Anomalies ◽  
Multiple Basal Cell Carcinomas

Gorlin syndrome is a rare autosomal dominant disorder exhibiting high penetrance and variable expressivity. It is characterized by facial dysmorphism, skeletal anomalies, multiple basal cell carcinomas, odontogenic keratocysts (OKC), palmar and plantar pits, bifid ribs, vertebral anomalies and a variety of other malformations. Various neoplasms, such as medulloblastomas, meningiomas, ovarian and cardiac fibromas are also found in this syndrome. Objective: To describe a twelve-year-old patient with Gorlin-Goltz syndrome,with basal cell carcinomas and promyelocytic leukemia developed after receiving craniospinal radiation for a medulloblastoma. Bifid ribs as well as mandibular and maxillar OKC were also diagnosed. Conclusion:The patient with Gorlin-Goltz syndrome should receive close follow-up for early detection of malformations and malignant neoplasias.

scholarly journals Late Onset of Multiple Basal Cell Carcinomas in a Patient with Gorlin-Goltz Syndrome Previously Treated for Hodgkin’s Lymphoma/ Kasna pojava multiplog bazocelularnog karcinoma kod pacijenta sa Gorlin-Golcovim sindromom prethodno lečenog od Hočkinovog limfoma

2014 ◽  
Vol 6 (1) ◽  
pp. 19-28
Author(s):  
Rumyana Yankova ◽  
Magdalina Gyurova ◽  
Donka Brambarova
Keyword(s):  
Basal Cell ◽  
Hodgkin’S Lymphoma ◽  
Late Onset ◽  
Hodgkin's Lymphoma ◽  
Goltz Syndrome ◽  
Basal Cell Carcinomas ◽  
Therapeutic Results ◽  
History Of ◽  
Multiple Basal Cell Carcinomas

Abstract Development of multiple basal cell carcinomas is commonly associated with immunosuppression or genetic disorders. The latter include congenital diseases such as Gorlin-Goltz syndrome, also known as nevoid basal cell carcinoma syndrome, or basal cell nevus syndrome. It is an autosomal dominant inherited disorder characterized by the development of multiple basal cell carcinomas at an early age and a variable combination of other phenotypic abnormalities that result in multiple organ involvement. The susceptibility gene was mapped to chromosome 9q22.3-3.1. Like other tumor suppressor genes, PTCH1 gene shows frequent deletion and a whole variety of other mutations. A high rate of new mutations and the variable expressivity of the condition make full diagnostic assessment difficult, especially in mildly affected individuals with no family history of the condition. It has been postulated that the presence of two major features or one major feature with two minor features classify a condition as Gorlin-Goltz syndrome. We present a 42-year-old male patient with a 6-year-long history of multiple smooth and/or rough skin patches and plaques on the back and shoulders. Some of the lesions gradually progressed and increased in number without any sensation. Dotlike, flesh-colored and brownish pits were found on the patient’s palms. Further investigations revealed many musculoskeletal and craniofacial congenital abnormalities such as pectus excavatum, frontal and parietal bossing, exotropia, ectopic teeth (impacted tooth), mandibular hyperplasia, broad nose. Histopathological examination by light microscopy of biopsies taken from the nodular and patchy skin lesions showed findings typical for basal cell carcinoma. Family history revealed no members with similar health disorders. The patient was treated for Hodgkin’s lymphoma with chemotherapy and radiation therapy 20 years before, with good therapeutic results, and no additional treatment was administered in the last ten years. The treatment for multiple basal cell carcinomas included: 5% imiquimod cream, 5 days a week, for 12 weeks. After 12 weeks of treatment, the nodular lesion and all the superficial lesions cleared. One month later the lesions disappeared completely without any residual signs. The patient was advised to use adequate photoprotection and to avoid future uncontrolled sun exposure. On follow-up visits during a three year period, no recurrent or new lesions indicative for BCC were seen. This is a case with late-onset multiple BCC in a patient with Gorlin-Goltz syndrome and a history of prior Hodgkin’s lymphoma. To the best of our knowledge hitherto only two cases of Hodgkin’s lymphoma in patients with Gorlin-Goltz syndrome have been reported in the literature. We also present therapeutic results of topical imiquimod for multiple basal cell carcinomas with no recurrent lesions over a three-year follow-up.

Follicular atrophoderma with multiple basal cell carcinomas. An abortive form of bazex syndrome.

1989 ◽  
Vol 51 (2) ◽  
pp. 250-255
Author(s):  
Seiji ARASE ◽  
Hideki NAKANISHI ◽  
Shin HARADA ◽  
Fumio SHIGEMI ◽  
Katsuyuki TAKEDA
Keyword(s):  
Basal Cell ◽  
Basal Cell Carcinomas ◽  
Multiple Basal Cell Carcinomas

scholarly journals Undifferentiated pleomorphic sarcoma in oropharyngeal mucosa of patients with multiple basal cell carcinomas

Rare Tumors ◽  
2021 ◽  
Vol 13 ◽  
pp. 203636132110264
Author(s):  
Andrea Dekanić ◽  
Marko Velepič ◽  
Margita Belušić Gobić ◽  
Ita Hadžisejdić ◽  
Nives Jonjić
Keyword(s):  
Differential Diagnosis ◽  
Basal Cell ◽  
Polypoid Lesion ◽  
Older Patient ◽  
Mesenchymal Tumors ◽  
Undifferentiated Pleomorphic Sarcoma ◽  
Pleomorphic Sarcoma ◽  
Basal Cell Carcinomas ◽  
History Of ◽  
Multiple Basal Cell Carcinomas

Malignant mesenchymal tumors of oropharyngeal mucosa are rare. Those with fibroblastic and histiocytic differentiation in the skin are called atypical fibroxanthoma (AFX) and in the soft tissue undifferentiated pleomorphic sarcoma (UPS). Here we present a case of an older patient with a history of multiple basal cell carcinomas and recently with a rapidly growing polypoid lesion in the mucosa of posterior oropharyngeal wall with AFX/UPS morphology. The differential diagnosis, histological pitfalls of this poorly characterized mesenchymal lesions, and the challenges associated with treatment are discussed.

Sonidegib in a patient with multiple basal cell carcinomas and HIV infection

2021 ◽  
Author(s):  
Vincent Hoffmann ◽  
Ralf Husak ◽  
Fritz Maiwirth ◽  
Bianca Sasama ◽  
Axel Zahn ◽  
...  
Keyword(s):  
Hiv Infection ◽  
Basal Cell ◽  
Basal Cell Carcinomas ◽  
Multiple Basal Cell Carcinomas
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