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Test Paper 7
Published in Teck Yew Chin, Susan Cheng Shelmerdine, Akash Ganguly, Chinedum Anosike, Get Through, 2017
Teck Yew Chin, Susan Cheng Shelmerdine, Akash Ganguly, Chinedum Anosike
Epidermoid cysts are also known as keratocysts. They are non-tender and usually palpable. The US appearance varies with the maturation, compactness and quantity of keratin present within the epidermoid cyst. A target appearance, a solid mass with an echogenic rim and a characteristic “onion ring” configuration with alternating layers of hyper and hypoechogenicity have been described. These cysts do not show blood flow at Doppler US examination. The constellation of an onion ring configuration, negative tumour marker status and avascularity help differentiate testicular epidermoids from other germ cell tumours.
The Classification of Odontogenic Cysts
Published in Roger M. Browne, Investigative Pathology of the Odontogenic Cysts, 2019
The most common radiological appearance is that of an apparent dentigerous cyst,9–11 being present in 40 to 50% of lesions. However, careful examination of the radiographs suggests that the crown of the associated unerupted tooth does not project into the cyst cavity, but rather has been displaced by it (Figure 2). This relationship is confirmed when the appropriate histological material can be examined.7,15 However, in a small proportion of cysts, the odontogenic keratocyst is in true dentigerous relationship to the associated unerupted tooth.7,16 This has given rise to the term follicular primordial cyst.17 There is evidence that such lesions arise originally in an extrafollicular position, discrete from the associated tooth, and subsequently either their epithelial linings fuse with the reduced enamel epithelium, so enclosing the entire anatomical crown of the tooth,7 or the tooth actually erupts into the cyst lumen.17 In whatever way the relationship arises, such cysts should be regarded as a variant of the odontogenic keratocyst and classified with them. In addition to its radiological appearance as a dentigerous cyst, the odontogenic keratocyst can assume a variety of other presentations, including that of a lateral periodontal cyst (Figure 3), a radicular cyst (Figure 4), a primordial cyst (Figure 1) and a residual cyst (Figure 5). It is this variety of clinical and radiological appearances that has contributed to the delay in the recognition of the odontogenic keratocyst as a distinct entity. A single case report of a primary odontogenic keratocyst arising solely in the alveolar mucosa has been published.18 The use of the term peripheral odontogenic keratocyst was suggested by the authors to describe this rare occurrence.
Concurrent basal cell carcinoma and tarsal epithelial cyst as a presenting sign of Gorlin syndrome
Published in Orbit, 2023
Meleha Ahmad, Rupin Parikh, Charles Eberhart, Roxana Fu
A 43-year-old Caucasian female presented with bilateral painless, slowly-progressing lower eyelid lesions. Past medical history was significant for two basal cell carcinoma (BCC) resections on her right cheek in the past 15 years. External exam photos are shown in Figure 1A,B. Shave biopsy of the right lower eyelid lesion and full thickness wedge biopsy of the left lower eyelid lesion demonstrated nodular BCC and tarsal epithelial cyst (TEC), respectively (Figure 1C,D). On further review of her records, she was found to have a previously unreported history of odontogenic keratocysts removed by her dentist on three occasions. Based on this constellation of findings, she met criteria for Gorlin syndrome and was referred to dermatology for further management. Gorlin syndrome, also called nevoid basal cell carcinoma syndrome, is a genetic condition characterized by widespread systemic manifestations including odontogenic keratocysts, multiple BCCs at an early age, and various developmental abnormalities and tumors. Although not part of the formal diagnostic criteria, Gorlin syndrome has also been associated with eyelid TECs, benign tarsal lesions that mimic chalazia. Recognition of this syndrome is important because these patients require close dermatologic surveillance and may benefit from treatment with vismodegib for advanced or widespread BCCs.
Nevoid basal cell carcinoma syndrome: a case report and literature review
Published in Ophthalmic Genetics, 2022
Shripadh Chitta, Jineet Patel, Shravan Renapurkar, Christopher Loschiavo, Jennifer Rhodes, Kayla King, Kimberly Salkey, Natario Couser
A very common finding seen in patients with NBCCS include recurrent multiple jaw cysts called keratocystic odontogenic tumors (KCOT) or odontogenic keratocysts (OKC). These are benign cystic neoplasms often found on X-Ray that have thin-walls and contain fluid and/or keratinaceous debris. Although traditionally classified under odontogenic cysts, the 2005 World Health Organization (WHO) classification placed the para-keratinized OKCs under the category of ‘benign neoplasm of odontogenic origin’ due to their locally aggressive behavior and higher tendency to recur. Again in 2017, the WHO reported lack of evidence to continue considering OKC as neoplasms despite acknowledging neoplastic behavior (33). The para-keratinized OKC/KCOTs are among the most common clinical manifestations seen in NBCCS and make up one of the major criteria for diagnosis. Prevalence ranges from 66% to 92% in studies (3,4,6,7,23,30,32). There is some evidence that suggests that they typically start presenting before the age of 18, with some studies reporting mean ages of first jaw cyst ranging from 15.5 to 19.8 years (6,7,32). Some studies even report first occurrence as young as 10 years old (3,6). Based on this information, the early presence of multiple OKCs in any patient should raise concern for possible NBCCS along with other notable symptoms. Sometimes this can be the first clinical and/or radiographic finding as a sign indicative of NBCCS as noted in our case report.
Advances in genetic understanding of gorlin syndrome and emerging treatment options
Published in Expert Opinion on Orphan Drugs, 2018
Shawn Shih, Christina Dai, Ahmed Ansari, Brittany A Urso, Amy I Laughlin, James A Solomon
Basal cell carcinoma nevus syndrome (BCCNS), or Gorlin syndrome, is an autosomal dominant syndrome that predisposes patients to tumor formation. It is characterized by basal cell carcinomas (BCC), odontogenic keratocysts (OKC, previously known as keratocystic odontogenic tumors), dyskeratotic palmar and plantar pitting, and skeletal and developmental abnormalities [1]. Patients also have characteristic facial features such as frontal bossing, macrocephaly, hypertelorism, and cleft lip/cleft palate [1,2]. The most prevalent feature of this syndrome is the development of BCCs at puberty, which are often more clinically aggressive than those seen in the general population [1,3]. Many patients, especially those with more UV light exposure or lighter skin, may develop hundreds of BCCs in their lifetime [2,4]. The most common location for BCC development is the face, followed by the trunk and limbs [2]. Patients with BCCNS also develop other neoplasms, such as desmoplastic medulloblastoma, meningioma, rhabdomyosarcoma, ovarian and cardiac fibromas, and mesenteric keratocysts [4,5]. Morbidity and premature mortality in BCCNS are primarily driven by development of neoplasms.