Original Article

Hiren P. Patt, MD1; Nikita Bothra, MBBS1; Atul H. Goel, Mch2; Rajeev Kasaliwal, DM1; Anurag R. Lila, DM1; Tushar R. Bandgar, DM1; Nalini S. Shah, DM1 ABSTRACT Objective: Limited data are available on pituitary gigantism, as it is a rare disorder. This study was carried out to assess the clinical, hormonal, and radiologic profiles and management outcomes of patients with pituitary gigantism. Methods: We conduced a retrospective analysis of 14 patients with pituitary gigantism who presented to a single tertiary care institute from 1990 to 2014. Results: Thirteen patients were male, and 1 was female. The mean age at diagnosis was 21.9 ± 6.1 years, with a mean lag period of 6.5 ± 5.6 years. The mean height SD score at the time of diagnosis was 3.2 ± 0.6. Symptoms of tumor mass effect were the chief presenting complaint in the majority (50%) of patients, while 2 patients were asymptomatic. Six patients had hyperprolactinemia. At presentation, the nadir PGGH (postglucose GH) and insulin-like growth factor (IGF 1)-ULN (× upper limit of normal) were 63.2 ± 94.9 ng/mL and 1.98 ± 0.5, respectively. All (except 1 with mild pituitary hyperplasia) had pituitary macroadenoma. Six patients had invasive pituitary adenoma. Transsphenoidal surgery (TSS) was the primary modality of treatment in 13/14 patients, and it achieved remission in 4/13 (30.76%) patients without recurrence over a median follow-up of 7 years. Post-TSS radiotherapy (RT) achieved

Submitted for publication January 6, 2015 Accepted for publication January 26, 2015 From the Departments of 1Endocrinology and 2Neurosurgery, Seth G.S. Medical College, KEM hospital, Mumbai, India. Address correspondence to Dr. Hiren P. Patt, Dept. of Endocrinology, Seth G.S. Medical College, KEM Hospital, Mumbai, India. E-mail: [email protected] Published as a Rapid Electronic Article in Press at http://www.endocrine practice.org on February 25, 2015. DOI:10.4158/EP15611.OR To purchase reprints of this article, please visit: www.aace.com/reprints. Copyright © 2015 AACE.

remission in 3/5 (60%) patients over a median follow-up of 3.5 years. None of the patients received medical management at any point of time. Conclusion: Gigantism is more common in males, and remission can be achieved in the majority of the patients with the help of multimodality treatment (TSS and RT). (Endocr Pract. 2015;21:621-628) Abbreviations: GH = growth hormone; GHRH = growth hormonereleasing hormone; IGF 1 = insulin-like growth factor 1; MEN 1 = multiple endocrine neoplasia type 1; PGGH = postglucose growth hormone; RT = radiotherapy; TSS = transsphenoidal surgery INTRODUCTION Classically, pathologic growth hormone (GH) excess causes acromegaly in adults, but in childhood or adolescence it leads to gigantism (1). There is an overlap between these 2 spectrums of GH excess. The majority of the patients with gigantism have subtle features of acromegaly (2), while 10% of the acromegaly patients may have tall stature (3). Pathological GH excess in childhood and adolescence is most commonly due to pituitary adenoma or rarely due to pituitary hyperplasia secondary to ectopic GH-releasing hormone (GHRH) excess or hypothalamic dysregulation. It can rarely occur as a part of multiple endocrine neoplasia type 1 (MEN 1)/ McCune-Albright syndrome/Carney complex (1). Pituitary gigantism is a rare disease with few reported case series to date (2,4-6). As early as in 1982, Whitehead et al (2) described marked skeletal deformity and psychological problems in patients with pituitary gigantism and addressed the need of early diagnosis and treatment to prevent the long-term sequelae of pathological GH excess. In the presomatostatin analog era, transsphenoidal surgery (TSS) and radiotherapy (RT) were the only options available to manage this disabling disease. Colao et al (4)

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622 Pituitary Gigantism, Endocr Pract. 2015;21(No. 6)

demonstrated the efficacy of depot somatostatin analogs in such patients. In the present study, we present the clinical, hormonal and radiologic profiles, as well as the longterm follow-up data of patients with pituitary gigantism managed with TSS and RT without medical management (somatostatin analogs and dopamine agonists). METHODS Methods We performed a retrospective analysis of patients with gigantism due to pathological GH excess who presented to a single tertiary care center. The study was conducted after approval from the institutional ethics committee. Gigantism was defined as height at diagnosis >97th percentile according to an Indian population growth chart (7). We managed 226 patients with pathologic GH excess at our department from 1990 to 2014. Fourteen (6.2%) patients were classified as giants. Diagnoses of pathologic GH excess were established by nadir postglucose GH (PGGH) value ≥1 ng/mL after a 75-g oral glucose tolerance test (GH levels were measured at every 30 minutes for up to 2 hours) and high IGF 1 levels for age and sex. Thorough clinical evaluations for syndromic associations of MEN 1, McCune-Albright syndrome (precocious puberty/café au lait spots) and Carney complex (skin pigmentation/ myxoma) were performed, but genetic analyses could not be undertaken. Patients were considered to have eunuchoid proportions if their upper/lower segment ratio was

PITUITARY GIGANTISM--EXPERIENCE OF A SINGLE CENTER FROM WESTERN INDIA.

Limited data are available on pituitary gigantism, as it is a rare disorder. This study was carried out to assess the clinical, hormonal, and radiolog...
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