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At least 19 recordsLinked to original sources
PubMed2005

Congenital melanotic macules and Sebaceous Choristoma arising on the tongue of a newborn: epidermal choristoma?

Oral hyperpigmentation is a common event in older individuals, however, is exceptional in neonates (congenital melanotic macules). Conversely, 70-80% of people have sebaceous glands in the oral mucosa, with the tongue representing an ectopic location and termed sebaceous choristoma by some authors. We report a case that fulfills both conditions in a tongue lesion. A 1-month-old boy presented with a pigmented macula on his tongue noted at birth. An excisional biopsy was performed showing a lesion lined by an epidermal-like epithelium with basal pigmentation, under which, sebaceous glands, abortive hair follicles and ductal structures mimicking apocrine glands were found. Seven cases of congenital melanotic macules of the tongue have been reported, however, none of them showed sebaceous glands under the lesion. Furthermore, there has not been a reported case of sebaceous choristoma of the tongue present at birth. We present a case that shares clinical and histological features of both conditions and propose the name 'epidermal choristoma'.

Choristoma↗
PubMed1999

Posterior scleral choristoma in the organoid nevus syndrome (linear nevus sebaceus of Jadassohn).

PURPOSE: To highlight the association of posterior osseous and/or cartilaginous ocular choristomas with epibulbar choristomas and the nevus sebaceus of Jadassohn. DESIGN: Small case series. PARTICIPANTS: Four patients with the organoid nevus syndrome. METHODS: Clinical and histopathologic studies in four patients with epibulbar lesions and nevus sebaceus of Jadassohn. MAIN OUTCOME MEASURES: Ophthalmoscopic findings of peripapillary lesions. Computed tomographic and ultrasonographic characteristic of posterior scleral lesions. Ocular histopathologic findings in one globe from one of the study subjects. RESULTS: Three patients had the triad of posterior osseous/cartilaginous ocular choristomas, anterior epibulbar choristomas, and nevus sebaceus of Jadassohn and one patient had anterior epibulbar choristomas and posterior osseous/cartilaginous ocular choristomas. Ultrasonography and computed tomography were valuable in detecting scleral ossification or epibulbar cartilage or both. The ophthalmoscopic findings were similar to those of a choroidal osteoma. CONCLUSIONS: The presence of posterior osseous/cartilaginous ocular choristomas in a patient with epilepsy or epibulbar lesions or both suggests the diagnosis of nevus sebaceus of Jadassohn. Osseous/cartilaginous ocular choristomas should be suspected in patients with nevus sebaceus of Jadassohn and peripapillary hypopigmented fundus lesions.

Cartilage↗
PubMed1998

Complex limbal choristomas in linear nevus sebaceous syndrome.

OBJECTIVE: This study aimed to describe the clinical and histopathologic findings in four patients with complex limbal choristomas associated with linear nevus sebaceous syndrome (LNSS), a rare disorder including nevus sebaceous, seizures, and mental retardation, and often accompanied by ocular anomalies. DESIGN: Small observational case series. METHODS: A retrospective review of the clinical and histopathologic records of four patients. RESULTS: Each of four patients had complex limbal choristomas in the setting of clinical and histopathologic LNSS. The limbal choristomas were multiple in three patients and bilateral in two patients. Most choristomas involved the superotemporal limbus (6 of 10), although nasal (3 of 10) and inferior (1 of 10) limbal tumors also were present. Three patients had significant corneal astigmatism or involvement of the central cornea requiring surgical removal of their choristomas, one accompanied by a lamellar keratoplasty and another accompanied by two consecutive penetrating keratoplasties. Each graft eventually vascularized, reducing vision. One patient's vision was limited by amblyopia and another by occipital cortical dysgenesis with visual impairment. Histopathologic examination of the excised choristomas showed foci of lacrimal gland (3 of 4 patients), adipose tissue (3 of 4), neural tissue (1 of 4), cartilage (1 of 4), lymphoid follicles (1 of 4), skin adnexal tissue (1 of 4), and smooth muscle (1 of 4). Other associated ocular findings included an eyelid mass (1 of 4), colobomas of the eyelid (3 of 4), colobomas of the choroid and retina (2 of 4), nonparalytic strabismus (2 of 4), scleral ectasia (1 of 4), partial oculomotor palsy with ptosis and anisocoria (1 of 4), microphthalmia (1 of 4), hypertelorism (1 of 4), and cortical visual impairment (1 of 4). CONCLUSIONS: Complex limbal choristomas, although rare, can occur in the setting of LNSS and can be associated with multiple ocular and systemic abnormalities. Visual prognosis appears poor in most cases despite aggressive management.

Child, Preschool↗
PubMedDate not supplied

Epibulbar osseous choristoma: case report and review of the literature.

Epibulbar osseous choristoma is a choristomatous lesion of the conjunctiva containing bone. Originally called epibulbar osteoma, this lesion was first described by von Graefe in 1863. We discuss a case of a 4-year-old patient who presented with an epibulbar lesion consistent histopathologically with an epibulbar osseous choristoma, and report a review of the literature. The differential diagnosis of osseous choristoma should include classical limbal dermoids, epithelial inclusion cysts, prolapsed orbital fat, papillomas, dermolipomas, and complex choristomas. Although the rarest of epibulbar choristomas, 51 epibulbar osteomas have now been reported in the medical literature. Most commonly, this variety of choristoma presents as an isolated epibulbar lesion within the supratemporal quadrant but may occur in other locations on the surface of the globe and possibly in conjunction with other choristomatous tissue as much as 10% of the time. Frequently, they may involve the muscle or have dense attachments to the underlying sclera. Osseous choristomas most likely represent congenital lesions with a potential for slow growth but may occur in association with trauma. Options for management include observation or surgical excision. When surgery is contemplated, preoperative radiographic imaging may be helpful for assessing adhesion to the sclera or extraocular muscles.

Bone and Bones↗
PubMed2006

Bilateral symmetrical epibulbar osseous choristoma.

PURPOSE: To report a patient with bilateral symmetrical epibulbar osseous choristoma. DESIGN: Retrospective observational case report. PARTICIPANT: One patient with bilateral symmetrical epibulbar osseous choristoma. METHOD: Retrospective review of stored office records, photographs, computed tomography (CT), and histopathological findings. MAIN OUTCOME MEASURES: Appearance of the choristoma, CT, and histopathological findings. RESULTS: A 6-year-old female had bilateral superotemporal subconjunctival masses. Surgical excision was performed bilaterally. The lesions were encapsulated and attached to the sclera, and consisted histopathologically of epibulbar osseous choristoma. CONCLUSION: We report the first case of bilateral symmetrical epibulbar osseous choristoma without other associated choristomas. Preoperative CT imaging is helpful in assessing the extent of the tumor and planning surgery.

Bone and Bones↗
PubMedDate not supplied

Intraocular choristoma.

Choristomas have been reported in the orbit, cornea, conjunctiva, uvea, retina, and optic nerve. Many reports have described the choristoma mainly in the epibulbar area, but little is known of the choristoma that occurs intraocularly. We reviewed the literature and summarized the reports that described the choristoma inside the eyes. Iris, ciliary body, choroid, and optic nerve head are the commonly affected tissues. In the anterior uveal tissue, an ectopic lacrimal gland is common and appears as a pinkish, nodular, fleshy mass. Its clinical course largely depends on the enlargement of the accompanying cyst. Osseous choristoma is mainly found in the choroid in young female patients. Although it is regarded as a benign tumor, it grows progressively and can decrease visual function. Treatment consists of close observation and possibly surgical intervention. Fortunately, most of the intraocular choristomas do not need aggressive treatment.

Animals↗
PubMed2004

Salivary gland choristoma of the middle ear. A review.

While head and neck surgeons are accustomed to recognizing malignancies in their practices, developmental lesions are much less often encountered, and as such are not as likely to be included amongst other (more aggressive) differential diagnostic considerations. One such developmental lesion is the choristoma, defined as an architecturally normal arrangement of mature tissues found in a location not normally host to such tissues. Choristomas composed of recognizable salivary gland tissue may be found in a variety of locations including the middle ear. Middle ear salivary choristomas are distinctly unusual lesions and typically manifest as unilateral conductive hearing loss; while both adult and pediatric patients have been diagnosed with middle ear choristomas, most patients have been in the first two decades of life. As these are benign, nonprogressive lesions, treatment has been directed toward complete surgical excision whenever feasible, and biopsy for diagnosis with subsequent observation in the case of those lesions which are difficult to remove without endangering the facial nerve. There have been rare instances in which choristomas have been linked to the subsequent development of neoplasms, but in the main choristomas are regarded as self-limited developmental heterotopias.

Choristoma↗
PubMedDate not supplied

Osseous choristoma of the oral soft tissue. Case report.

Oral osseous choristoma is a rare developmental alteration, their principal localization is nearly to base of the tongue (foramen caecum). The oral mucosa localization of osseous choristoma is extremely rare. At date only 10 cases of oral mucosa osseous choristoma had been reported. In the present paper we reported a new case of oral mucosa osseous choristoma in a Klippel-Feil syndrome patient. A review of available literature was made. We presented a 28 years old female patient who showed into the right oral mucosa, a hard, mobile, and asymptomatic mass, with minimum 4 years of evolution. The histological image showed a lesion constituted by lamellar bone, osteocytes and haematopoyetic tissue. The diagnosis of osseous choristoma was made. It is discusses their possible association with Klippel-Feil syndrome. The osseous choristoma of buccal mucosa is most frequently in fifth decade of the life although is reported between 12-to-64 years old, with a female predisposition.

Adult↗
PubMedDate not supplied

Ocular choristomas.

Choristomas are congenital lesions representing normal tissue(s) in an abnormal location. They are the most common epibulbar and orbital tumors in children. Epibulbar choristomas affect the cornea, limbus or subconjunctival space, and range in appearance from a small, flat lesion to a large mass filling most of the epibulbar region. Astigmatism is often present. Choristomas may be associated with coloboma, Goldenhar syndrome or epidermal nevus syndromes; those associated with the latter are often bilateral and extensive. Choristomas are occasionally familial. Surgery may be indicated to improve vision or cosmesis, or to impede growth. Although choristomas most commonly involve the epibulbar area, they can affect many areas of the eye and orbit, and often affect more than one area.

Astigmatism↗
PubMed1993

Phakomatous choristoma (Zimmerman's tumor). Immunohistochemical confirmation of lens-specific proteins.

BACKGROUND: Phakomatous choristoma is a rare, congenital, ocular adnexal tumor that is presumed to be of lenticular anlage based on light and electron microscopy. METHODS: The authors performed immunohistochemistry using standard commercially available antibodies against vimentin, S-100 protein, and several cytokeratins on a phakomatous choristoma that was excised from the right lower eyelid of a 10-week-old white boy. In addition, a battery of antibodies against lens-specific proteins, including alpha, beta, and gamma crystallins, was used. RESULTS: The tumor cells showed intense immunoreactivity for all lens-specific proteins tested. The epithelial cells of the phakomatous choristoma stained positively for S-100 protein and vimentin, the intermediate filament normally found in lens epithelial cells. Keratin markers were negative. CONCLUSIONS: The results of immunohistochemistry indicate that the cells of phakomatous choristoma synthesize several types of lens-specific proteins. Complementing previous light and electron microscopic studies, these data strongly support Zimmerman's conclusion that this pediatric adnexal tumor is a choristoma of lenticular anlage.

Antibodies, Monoclonal↗
PubMed1999

Phakomatous choristoma may be located in the eyelid or orbit or both.

PURPOSE: Phakomatous choristoma is a rare congenital tumour of lenticular origin. The exact location at clinical presentation is controversial, but herein we provide evidence that phakomatous choristoma may be located in the lower eyelid or orbit or both. METHODS: Case report of an infant presenting with a mass in the lower eyelid at birth, a systematic review of previous cases of phakomatous choristoma and an outline of the histopathological features in normal mid-facial embryonic development at different gestational ages. RESULTS: The histopathological features of the present case were consistent with those of a phakomatous choristoma. The preoperative imaging studies and clinical findings at surgery suggested that the tumour occupied parts of both the lower eyelid and anterior orbit. Histopathological sections of a normal human embryo showed that at the 26-mm stage of development the embryonic lens is formed but the bony walls defining the orbit are not yet present. CONCLUSION: Phakomatous choristoma arises in a setting of undifferentiated mesenchymal tissue which later may develop into the lower eyelid or orbit depending on the choristomatous elements being deposited superficial or deep to the embryonic surface.

Avidin↗
PubMed2001

Choristoma of the middle ear: a component of a new syndrome?

CASE REPORT: Salivary choristoma of the middle ear is a rare entity. The authors report the 26th known case, which is unique in several respects: the patient had abnormalities of the first and second branchial arches, as well as the otic capsule and facial nerve in ways not yet reported. Our patient presented with bilateral preauricular pits, conchal bands, an ipsilateral facial palsy, and bilateral Mondini-type deformities. A review of the literature revealed salivary choristomas of the middle ear to be frequently associated with branchial arch abnormalities, most commonly the second, as well as abnormalities of the facial nerve. REVIEW OF THE LITERATURE: All 25 cases were reviewed and the results reported with respect to clinical presentation, associated abnormalities, operative findings, and hearing results. It has been proposed that choristoma of the middle ear may represent a component of a syndrome along with unilateral hearing loss, abnormalities of the incus and/or stapes, and anomalies of the facial nerve. CONCLUSION: Eighty-six percent of the reported patients with choristoma have three or four of the four criteria listed to designate middle ear salivary choristoma as part of a syndrome. In the remaining four patients, all of the structures were not assessed.

Child↗
PubMed1992

A case of amniotic band syndrome with bilateral epibulbar choristoma.

An autopsy case of amniotic band syndrome with bilateral epibulbar choristoma is described. The left eye reveals a complex choristoma and the right eye a dermis-like choristoma. Both choristomatous lesions included lenticular tissue suggesting that rupture of the amnion, which is the initial event of amniotic band syndrome, might have occurred at about the fourth week of gestation. Since the other systemic manifestations of amniotic band syndrome are considered to be compression deformities of the fetus caused by oligohydramnios or amniotic band, the occurrence of epibulbar choristomas in both eyes in this case suggests that a compression mechanism may play a role in the pathogenesis of epibulbar choristoma.

Adult↗
PubMed2003

[Epibulbar osseous choristoma: two case reports].

INTRODUCTION: Epibulbar osseous choristoma is a congenital tumor consisting of normal tissue arising in an abnormal location. CARE REPORTS: We report two cases of epibulbar osseous choristoma discovered in 7- and 3-year-old girls. FIRST CASE: A calcified tumor was located regarding the temporal bulbar conjunctiva of the left eye. The rest of the ophthalmological examination was normal. Second case: During strabismus surgery, we unexpectedly discovered a white calcified mass located on the superior temporal part of the right eye. In both cases, a total excision was performed. Histopathological examination of the excised choristoma confirmed the diagnosis. DISCUSSION: Epibulbar osseous choristoma is a small unique nodule usually located on the superior temporal quadrant of the eye. Rarely reported in the literature, most cases are not recognized clinically and the diagnosis is essentially histopathological. CONCLUSION: Epibulbar osseous choristoma is a rare benign childhood tumor with an essentially histopathological diagnosis.

Bone and Bones↗
PubMed1998

Lingual osseous choristoma: a study of eight cases and review of the literature.

A choristoma is a tumor-like mass of normal tissue in an "abnormal" location. Lingual osseous choristoma, previously known as osteoma of the tongue, is a rare entity: we found documentation of eight cases at our center during the 10-year period between 1987 and 1996. These cases were analyzed along with 50 others reported in the English language literature between 1913 and 1996. Lingual osseous choristoma frequently occurs during the third and fourth decades of life, and predominantly affects women (81%). Most of the osseous choristomas in our review were located in the posterior third of the tongue, primarily at or close to the foramen cecum or circumvallate papillae (87.9%). While 39.7% of the patients were asymptomatic, the remaining patients complained of symptoms including a lump in the throat (25.8%), dysphagia (6.9%), gagging (5.1%), nausea (3.4%) and irritation (3.4%). Treatment of lingual osseous choristoma consists of simple excision. The tumor's origin has been discussed elsewhere.

Adult↗
PubMedDate not supplied

Lacrimal gland choristoma of the ciliary body.

BACKGROUND: Lacrimal gland choristoma manifesting in the ciliary body is quite rare. We report a case of lacrimal gland choristoma manifesting in the ciliary body, coupled with orbital cellulites, in an infant. CASE: A 10-month-old female infant with swelling and tenderness of the right upper eyelid. OBSERVATIONS: The results of ophthalmic examinations of the patient were consistent with orbital cellulitis. During the funduscopic examination, a fleshy mass was incidentally detected at the superotemporal portion of the ciliary body in the right eye. A local resection of the lesion was carried out. The results of a pathologic examination showed lacrimal gland choristoma of the ciliary body that was accompanied by an epithelia-lined cyst. The patient has had no hypotonia or enlargement of the lesion for 1 year after surgery. CONCLUSION: Lacrimal gland choristoma should be included in the differential diagnosis of a ciliary body mass in pediatric patients.

Biopsy↗
PubMed2005

Optic nerve choristoma.

Choristomas are rare lesions that typically involve peripheral nerves and are marked by the presence of histologically normal tissue for an organ or body part other than the site at which it is located. Reports of choristoma involving cranial nerves are extraordinarily rare. We report a case of an optic nerve choristoma arising in a 24-year-old woman. The lesion was marked by a disordered admixture of smooth muscle tissue and optic nerve tissue. The literature on optic nerve choristoma is reviewed.

Actins↗