The basal cell nevus syndrome is a well recognized entity, the major symptoms of which are basal cell nevi, multiple jaw cysts, skeletal anomalies, and ectopic calcification. The syndrome follows a hereditary pattern, which is characterized by a highly penetrant, autosomal dominant gene with multiple and variable effects. The patient often has a characteristic face, with frontal and temporoparietal bossing, which results in an increased cranial circumference. The eyes may appear widely separated, and 40 percent of patients have true ocular hypertelorism. Jaw cysts are one of the most constant features of the syndrome and are present in at least 75 percent of the patients. The cysts are odontogenic keratocysts and frequently multiple. Radiographically, the cysts in patients with basal cell nevus syndrome do not differ significantly from isolated keratocysts. The cysts in patients with this syndrome are often associated with the crowns of unerupted teeth; on radiographs they may mimic dentigerous cysts. We report a case of multiple odontogenic keratocysts associated with basal cell nevus syndrome with the literature of review.
Basal Cell Nevus Syndrome* ; Crowns ; Dentigerous Cyst ; Genes, Dominant ; Humans ; Hypertelorism ; Jaw Cysts ; Nevus ; Odontogenic Cysts* ; Tooth, Unerupted

Nevoid basal cell carcinoma syndrome is a genetically determined disorder characterized by multiple basal cell carcinoma, palmoplantar pits and defects in other organs or tissues. Basal cell carcinoma may arise any sit of body. But infrequently typical basal cell carcinomas arise from the depressed epidermis which forms the base of the pits on the hands and feet. The patient was a 12 years old girl who developed multiple black-colored papules on the face and a dark reddish to black-colored pit over the palm and skin-colored depressed circumscribed pits on the plantar surface of feet. she also had an odontogenic cyst in the right maxilla, epidermal cyst on the trunk and mutiple intradermal nevi on the face. But the family history was not present. The histopathologic findings of the depressed pit of the palm showed a crater on the stratum corneum, and a well marginated tumor nest of basal cell carcinoma showing keratotic feature on the center of the lesion and reticulated feature on the periphery.
Basal Cell Nevus Syndrome* ; Carcinoma, Basal Cell* ; Child ; Epidermal Cyst ; Epidermis ; Female ; Foot ; Hand ; Humans ; Maxilla ; Nevus, Intradermal ; Odontogenic Cysts

Malignant cystic lesions of the lateral side of the neck are usually due to metastasis and are rarely primary carcinoma. The most common is metastatic cystic squamous cell carcinoma arising from a primary site at the Waldeyer's ring. However, they can arise from papillary carcinoma of thyroid, lung and mediastinum but very rarely from the abdominal and pelvic organs without regional lymph node involvement. Therefore, accurate diagnosis is very important for appropriate management. We report two cases of cystic squamous cell carcinoma of the lateral neck; cystic metastasis from an occult squamous cell carcinoma of the tongue and a primary branchiogenic carcinoma. We emphasize the importance of meticulous search for the primary malignant lesions in cases of cystic carcinoma of the neck.
Branchioma ; Carcinoma ; Cysts ; Metastasis

We present two cases of multiple jaw cysts not associated with basal cell nevus syndrome. Case 1: a nine year-old boy visited CNU Hospital for orthodontic treatment and his radiographs showed cystic lesions surrounding the crowns of teeth #13 and #17 respectively, which were diagnosed as dentigerous cysts. Subsequently, two more cysts were found on his follow-up radiographs in 12 and 15 months. The two cysts were determined to be odontogenic keratocysts. The boy had no skeletal abnormalities and no skin lesions associated with basal cell nevus syndrome. Case 2: a fifty-eight year old man had three impacted third molars with pericoronal radiolucencies, which were diagnosed as dentigerous cysts. He had no additional abnormalities associated with basal cell nevus syndrome. Multiple jaw cysts can occur at any age, and periodic radiographic surveillance may be needed for any cases of impacted tooth.
Basal Cell Nevus Syndrome* ; Crowns ; Dentigerous Cyst ; Follow-Up Studies ; Humans ; Jaw Cysts* ; Jaw* ; Male ; Molar, Third ; Odontogenic Cysts ; Skin ; Tooth ; Tooth, Impacted

On H1 MRS (magnetic resonance spectroscopy), malignant tumors show higher concentration of metabolite than benign lesions. Lactate double peak was detected in malignant tumor and endometriosis, and more prominent high concentration was demonstrated in endometriosis. Tuboovarian abscesses and salpingitis do not show prominent peak. Dermoid cysts show high levels of lipid peak. Paratubal cyst and follicular cyst can be showed the lipid peak, however, the concentration of lipid is lower than that of dermoid cyst. H1 MRS of ovarian cystic lesions can give valuable information about the presence of metabolites of ovarian cystic lesions.
Abscess ; Dermoid Cyst ; Endometriosis ; Female ; Follicular Cyst ; Lactic Acid ; Magnetic Resonance Spectroscopy* ; Ovarian Cysts ; Ovary ; Parovarian Cyst ; Salpingitis

Dentigerous cysts are benign odontogenic cysts that are associated with the crowns of permanent teeth. They are developmental epithelial-lined lesions which are formed from fluid accumulation in the space between the reduced enamel epithelium and the surface of crown. This may occure due to the obstructin of venous return caused by tooth impaction. The most prevalent location of dentigerous cysts are the third molar of mandible. While the single dentigerous cysts are the second most common odontogenic cysts following the radicular cysts of jaw, multiple cysts are observed in patients with some conditions such as mucopolysaccharidosis type IV, basal cell nevus syndrome, and cleidocranial dysplasia. They occur in young patients in the second or third decades of life, but it is a rare occurrence for children. A sixteen-year-old girl with painful swelling in the right mandible visited to our department. All routine laboratory test results were within normal limits. However, we were able to diagnose that she had facial asymmetry. Computed tomography showed a well-defined soft tissue mass obliterating right mandible ramus with an impacted tooth. We performed total enucleation of cyst and molar tooth. The pathology revealed a non-keratinized squamous epithelial lined cyst associated with an undamaged tooth.
Basal Cell Nevus Syndrome ; Child ; Cleidocranial Dysplasia ; Crowns ; Dental Enamel ; Dentigerous Cyst* ; Epithelium ; Facial Asymmetry ; Female ; Humans ; Jaw ; Mandible* ; Molar ; Molar, Third ; Mucopolysaccharidoses ; Odontogenic Cysts ; Pathology ; Radicular Cyst ; Tooth ; Tooth, Impacted

The basal cell nevus syndrome (BCNS) is an autosomal dominant disorder, characterized by basal cell carcinomas, odontogenic keratocysts and skeletal abnormalities. We experienced two cases that represented several characteristics of BCNS. Case 1: a thirty three year-old man visited CSU hospital. His radiographs showed four cystic lesions at both maxillary sinus and both mandibular angle, with bifid rib and ectopic calcification of falx cerebri. After marsupialization and enucleation, recurrent and newly developing tendency were found on his follow-up radiographs. Case 2: a seventeen year-old man had four large cystic lesions which were diagnosed as odontogenic keratocysts. He had craniofacial anomalies which included ectopic calcification and frontal bossing.
Basal Cell Nevus Syndrome ; Follow-Up Studies ; Jaw Cysts ; Nevus ; Odontogenic Cysts ; Ribs ; Tooth, Impacted

Basal cell nevus syndrome (BCNS) is principally characterized by cutaneous basal cell carcinomas, multiple odontogenic keratocysts and skeletal abnormalities. Our patient represented several characteristics of BCNS, such as, multiple odontogenic keratocysts, facial nevus, calcification of falx cerebri, parietal bossing and mental retardation. The cyst on posterior mandible showed recurrent and newly developing tendency.
Basal Cell Nevus Syndrome* ; Carcinoma, Basal Cell ; Humans ; Intellectual Disability ; Mandible ; Nevus ; Odontogenic Cysts* ; Rabeprazole

Basal cell nevus syndrome(BCNS) is a rare autosomal dominant disorder characterized by variable developmental anomalies and predisposition to cancers. The main manifestations include multiple basal cell carcinomas of skins, odontogenic keratocysts, facial dysmorphism, skeletal abnormalities including scoliosis and bifid ribs, palmar and plantar pits, calcification of the falx cerebri, and biparietal frontal bossing. We experienced a case of 12-year-old girl with the clinical features of basal cell nevus syndrome.
Basal Cell Nevus Syndrome ; Carcinoma, Basal Cell ; Child ; Humans ; Nevus ; Odontogenic Cysts ; Ribs ; Scoliosis ; Skin

The combination of melanocytic nevus with other tumors of epidermal or adnexal origin has been described since the first case reported by Andresen and Enos in 1952. Most of reported cases are melanocytic nevi combined with common epidermoid cysts. The combination of nevus and cyst could possibly be misdiagnosed clinically as a malignant melanoma upon initial examination. A 58-year-old woman presented with an asymptomatic, solitary, skin-colored, erythematous 1.0 cm-diameter pedunculated nodule on the occipital scalp which had been present childhood. The histopathologic specimen of the nodule showed a solitary cyst surrounded by intradermal melanocytic nevus. The cyst was located in the dermis lined by squamous epithelium, which was ruptured and inflamed. It contained degenerated keratin materials and multiple transversely and obliquely cut vellus hairs. We herein report a rare case of melanocytic nevus combined with a vellus hair cyst.
Bone Cysts ; Dermis ; Epidermal Cyst ; Epithelium ; Female ; Hair* ; Humans ; Melanoma ; Middle Aged ; Nevus ; Nevus, Pigmented* ; Scalp