Pituitary Duplication Research Articles

Finding its place on the spectrum of pituitary duplication disorders, duplication of pituitary stalk: A case report with brief review of literature.

Pituitary gland duplication is a rare abnormality and isolated duplication of the pituitary stalk without any pituitary anomaly is an even rarer entity with this case being the first documented case till date. Although incidentally discovered cases of duplication of pituitary gland (DPG) have been reported, sometimes with a duplicated pituitary stalk, patients with this disorder usually present with other craniofacial abnormalities. Consequently, DPG plus syndrome is used as it is often accompanied by endocrine disturbances and pathologies such as median cleft face syndrome, ocular disorders, craniocervical bony abnormalities, vascular anomalies and tuberomammillary masses. Since this is the first reported case without any additional pituitary gland anomaly, we propose the acronym DPS (duplication of pituitary stalk) to be used to unify this entity as we are certain that much like the previously described pituitary duplication disorders, more cases will be documented independently rather than under the umbrella of pituitary duplication disorders. This is critical as the life expectancy (age of diagnosis) in the cases reviewed in our study is as good as normal population with no obvious increase in mortality as compared to existing pituitary duplication syndromes. We present a case report of a 2year 7month old girl who was referred by the paediatrician for evaluation of premature thelarche. The duplication of the pituitary stalk along with mega cisterna magna and tuberomammillary fusion was the only positive finding on imaging with the pituitary gland being absolutely normal.

Isolated pituitary stalk duplication Pituitary stalk duplication

Isolated pituitary stalk duplication Pituitary stalk duplication

Pituitary Duplication Presenting as Airway Obstruction: A Case Report

Pituitary Duplication Presenting as Airway Obstruction: A Case Report

Duplication of pituitary gland: A rare congenital anomaly

Pituitary duplication is a rare malformation, with only a few previously reported cases. It occurs most commonly in association with other complex midline craniofacial abnormalities. We present the imaging findings of a 1-year-old girl who has been diagnosed coincidentally by magnetic resonance imaging with an isolated pituitary gland duplication but no other major craniofacial malformations.

Nasal Hamartoma Associated With Duplicated Pituitary

Nasal hamartomas are rare congenital lesions. We describe a case of nasal hamartoma associated with pituitary duplication and other midline anomalies. A 40-year-old female with a history of breast cancer presented with nasal obstruction. Computed tomography and magnetic resonance imaging revealed a mass arising from the nasal septum, as well as duplication of the pituitary and a skull base canal that extended from the margin of the left pituitary fossa to the nasal mass. The mass was subsequently resected via a transnasal endoscopic approach and histology confirmed the presence of hamartoma. Nasal hamartomas are benign lesions that can be associated with other midline anomalies, such as duplicated pituitary, and can be managed conservatively.

Duplicated Pituitary Gland and Odontoid Process

The development of the pituitary gland is not well understood, but duplication of the gland, a rare embryonic anomaly, may shed some light on the process. Since 1880 only about 40 cases have been described. A 56-year-old woman complained of chronic bilateral upper extremity paresthesia and numbness along her first three fingers relieved by rest and exacerbated by increased activity. Magnetic resonance imaging of her head and neck showed an incidental discovery of a duplication of the pituitary infundibulum and pituitary fossa. Computed tomography of the neck showed congenital fusion of C2 with C3, C4 with C5, C1 with the occipital bone, and a duplication of the odontoid process. Her physical examination and all laboratory data were negative. Only seven patients with a pituitary duplication have ever survived beyond puberty. While all of these patients had normal mental capabilities, they also all had obvious craniofacial malformations. Unlike our patient, all other reported cases of duplicated pituitaries have been associated with abnormalities of the face or brain. Previously proposed theories for duplicated pituitary glands include failed twinning, teratogens, and an extreme form of the median cleft face syndrome. We feel that the cleft theory developed by Morton et al. best describes the cause of our patient's malformations. Such anomalous presentations will improve our understanding of how the pituitary gland develops and the order in which cranial structures develop to cause these cranial malformations.

A Female Infant Who Has a Pink, Smooth Soft-Tissue Mass

A female newborn has a pink, fresh, smooth, soft tissue mass (2.0 cm x 1.5 cm x 1.0 cm) on the midline of the hard palate (Fig 1).Serum alpha feto protein was 61,575 ng/mL.Congenital epulisEpulis is a benign tumor arising from the gingival mucosa. It is also known as congenital gingival granular cell tumor or Neumann’s tumor after its first description in 1871.This gingival granular cell tumor grows only in utero, especially during the third trimester of gestation. No known cause of epulis has been identified though it has been theorized that intrauterine estrogen stimulates its development because of its female predominance and spontaneous regression once maternal estrogen is withdrawn.Epulis is rare. Only 186 published cases were found in a literature review by Yavuzer in 2001. It is more common in females, with female-to-male ratio from 3:2 to 10:1. The tumor occurs more commonly from the maxilla than the mandible, with 2:1 ratio. It involves both maxilla and mandible in 10% of cases. Our case is unusual in that it appeared on the palate.The lesion is typically a pink, smooth-surfaced, soft tissue mass attached to the gingiva by a broad base or by a pedicle. Its size is from 1 mm to 9 cm in diameter. The infant with epulis may have feeding problems and respiratory difficulties due to airway obstruction. Polyhydramnios secondary to a blockage of the fetal oral cavity and inability to swallow amniotic fluid can occur.The diagnosis of epulis can be made based on clinical findings and it is confirmed histologically. On histological examination, epulis has sheets of round cells with granular cytoplasm and small nonmitotic nuclei, which are surrounded by a delicate stroma of collagen and covered by the squamous epithelium with no pseudoepitheliomatous hyperplasia. It tends to have a prominent plexiform network of capillaries. Epulis may be diagnosed by prenatal ultrasonography.Simple surgical resection of the tumor is required. Our case reveals that some cases can spontaneously regress over time without resection. Radical excision is discouraged because of the damage to underlying structures. There are case reports that reveal the conservative approach to the small asymptomatic lesion can succeed in spontaneous regression. No recurrence or malignant transformation has been reported.Epignathus, a congenital oropharyngeal teratoma is one of the differential diagnoses. It originates from the craniopharyngeal canal and attaches to the base of the skull in the posterior region of the nasopharynx. Epignathus is a malignant tumor and is difficult to distinguish from an epulis on clinical grounds alone. Few cases with an intracranial extension along the craniopharyngeal canal can be fatal. The infant with epignathus may also have other malformations, such as cleft palate, bifid tongue, bifid nose, pituitary duplication, and hypoplasia of the corpus callosum.Because there were no feeding or respiratory problems, management was conservative, and the infant was discharged. The epulis underwent spontaneous regression by the time the infant was 2 months old.JoDee M. Anderson, MD, MEd, Division of Neonatal Medicine, Oregon Health & Science University, Portland, ORSpecial acknowledgements are given to Dr Art Cooper, Department of Pediatric Surgery; Dr Jason Moche, Department of ENT; and Dr Cesar Ong, Department of Radiology, Harlem Hospital Center, New York, NY.

Pituitary duplication: a rare cause of precocious puberty

Pituitary duplication: a rare cause of precocious puberty

Pituitary Duplication With Nasopharyngeal Teratoma and Cleft Palate

Pituitary gland duplication is a rare malformation of unknown cause that is often associated with a nasopharyngeal teratoma, among other secondary malformations. This clinical report describes a case of pituitary gland duplication with a nasopharyngeal teratoma, cleft palate, and hypothalamic hamartoma, as well as the surgical management of this patient. This case also raises the question of whether the nasopharyngeal teratoma is the cause of the pituitary duplication above and the cleft palate below or whether it is a result of the primary duplication of the notochordal process. Various theories are presented in an attempt to answer this question, but the exact cause of these malformations remains equivocal. Future research in this topic may elucidate the answer to this question.

Pituitary dysfunction in an adult caused by duplication of the pituitary gland

Pituitary duplication is a rare malformation reported previously in approximately 34 patients. Survival beyond infancy is normally not observed due to various congenital malformations. We describe a case of a 30-year-old man who presented with secondary hypogonadism and gynaecomasty. MR imaging delineated two small pituitary glands and a large persistent craniopharyngeal canal. No other congenital malformations were present. It is evident that the duplication of the pituitary gland can occur without other major dysmorphias.

Central Precocious Puberty Associated with Pituitary Duplication and Midline Defects

Central precocious puberty (CPP) is due to premature activation of the hypothalamic-pituitary-gonadal axis. It may be idiopathic or result from congenital or acquired CNS lesions. We describe a 7.4 year-old Brazilian girl with CPP who also presented hypertelorism, limitation of lateral neck rotation and synkinesis of the hands. Sellar and cervical column MRIs revealed pituitary duplication and rudimentary intervertebral disks. We present the clinical and imaging observations of this case, and a thorough literature review of this rare developmental abnormality.

Pituitary duplication and nasopharyngeal teratoma in a newborn: CT, MRI, US and correlative histopathological findings

The computed tomography and MRI imaging findings in a case of pituitary duplication and epipharyngeal teratoma are described in a newborn baby girl with respiratory difficulties. Associated skull base and central nervous system malformations are presented. Teratoma diagnosis was confirmed by histology. The embryological pathogenesis is discussed.

Duplication of the hypophysis associated with precocious puberty: presentation of two cases and review of pituitary embryogenesis

Pituitary duplication is a rare malformation commonly associated with other major neural/craniofacial anomalies, easily shown by magnetic resonance imaging. The authors describe two girls with duplication of the pituitary gland and thickening of the hypothalamus, facial dysmorphism and precocious pubertal development. The pathogenesis of pituitary duplication and its relationship with precocious pubertal development are discussed.

Case 79: Pituitary Duplication

Case 79: Pituitary Duplication

Pituitary duplication associated with oral dermoid and corpus callosum hypogenesis

We report a case of pituitary duplication in a neonate girl whose magnetic resonance (MR) images showed unusual findings of hypogenesis of the corpus callosum and oral dermoid. Pituitary duplication is an extremely rare malformation, with only a few previously reported cases. It occurs most commonly in association with complicated midline and skull base anomalies. We present a case of this malformation with special emphasis on the hypogenesis of splenium of the corpus callosum and oral dermoid.