a difficult to treat case of cushing s syndrome

MRI of pituitary showed a focal hypodense lesion and CT adrenal showed hyperplasia of right adrenal remnant [Figure 1].. Her cortisol levels done Figure 1: CT scan abdomen showing hyperp

Letters to the Editor

Indian Journal of Endocrinology and Metabolism / Jan-Feb 2013 / Vol 17 | Issue 1 181

Corresponding Author: Dr Amitabh Sagar,

Department of Internal Medicine, Armed Forces Medical College, Pune, Maharashtra, India

E‑mail: amitthephysician@gmail.com

refereNCes

1 Feroze M, Arvindan KP, Jose L Gaucher’s disease among Mappila

Muslims of Malabar Indian J Pathol Microbiol 1994;37:307‑11.

2 Beutler E, Grabowski GA Glucosylceramide lipidosis–Gaucher

disease In: Scriver CR, Beaudet AL, Sly WS, Valle D, editors

The Metabolic and Molecular Bases of Inherited Diseases 8 th ed

New York: McGraw‑Hill; 2001 p 3635‑68.

3 Ginsberg H, Grabowski GA, Gibson JC, Fagerstrom R, Goldblatt J,

Gilbert HS, et al Reduced plasma concentrations of total, low density

lipoprotein and high density lipoprotein cholesterol in patients with

Gaucher type I disease Clin Genet 1984;26:109‑16.

4 Bohra V, Nair V Gaucher’s disease Indian J Endocrinol Metab

2011;15:182‑6.

5 Grabowski GA Phenotype, diagnosis and treatment of Gaucher

disease Lancet 2008;372:1263‑71.

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A difficult to treat case

of Cushing’s syndrome

Sir,

An 18‑year old girl presented in 1998 with clinical,

biochemical, hormonal and radiological evaluation

consistent with Cushing’s disease She underwent

trans‑sphenoidal resection for pituitary microadenoma

Serum cortisol level on third post‑operative day remained

elevated She was re‑evaluated in June 2001 and underwent

bilateral adrenalectomy in May 2002 She had marked

improvement in her symptoms and was on steroid

replacement In March 2003 her symptoms re‑appeared

and glycemic control deteriorated She has already stopped

steroid replacement Her basal cortisol done twice was

elevated (32 and 38 µg/dl) with raised ACTH levels

(98.7 pg/ml) MRI of pituitary showed a focal hypodense

lesion and CT adrenal showed hyperplasia of right adrenal

remnant [Figure 1] She underwent host of imaging studies

to find missed ectopic ACTH secreting tumor, but were

negative She was advised second trans‑sphenoidal surgery

for which she refused She was started on tab ketoconazole,

which she took intermittently Her cortisol levels done

Figure 1: CT scan abdomen showing hyperplasia of right adrenal remnant

every year, remained elevated Gamma knife instrument was installed in this hospital in 2007 In 2010, we persuaded her to undergo gamma‑knife surgery, which she refused However, on repeated persuasion she agreed, and underwent

it in September 2011 After surgery her symptoms gradually improved In July 2012, she reported with persistent anorexia, vomiting, extreme asthenia with weight loss

of about 20 kg Examination revealed hypotension and investigations showed hyponatremia Na‑129 meq/l, hyperkalemia K‑6.0 meq/l and hypoglycemia Her insulin requirement had come down by 50% with no need for anti‑hypertensive, and her cortisol was 2.6 µg/dl Finally she had developed adrenal insufficiency with possible permanent cure of Cushing’s disease

This case highlights agony of patient and treating physician She had first failed pituitary surgery indicated by persistent hypercortisolemia She had recurrence even after bilateral adrenal surgery Recurrence after adrenalectomy may be due either to regrowth of adrenal cells in the surgical bed or growth of adrenal rest tissue Unsuccessful adrenalectomy

is usually the consequence of failure to excise hyperplastic adrenal tissue extending around the right adrenal vein.[1] At this stage, a missed or occult ectopic‑ACTH secreting tumor was suspected Common causes of such slow growing tumor are thoracic carcinoid (36‑49%), small cell carcinoma of lung (18‑37%) and others (15‑44%) like pancreatic carcinoid, pheochromocytoma and medullary thyroid carcinoma.[2] Her imaging studies for ectopic‑ACTH secreting tumor were negative Facilities for inferior petrosal sinus sampling were not available at this hospital at that time Options

at his stage were second TSS, remnant adrenalectomy, radiotherapy or medical therapy Success rate of second TSS varies between 30 to 70% and predictors of success are correct diagnosis, initial incomplete TSS and residual tumor on CT/MRI However, in absence of biochemical evidence such focal defects in the pituitary gland on CT/

Letters to the Editor

Indian Journal of Endocrinology and Metabolism / Jan-Feb 2013 / Vol 17 | Issue 1

182

MRI should not be taken as absolute evidence of presence

of a corticotroph adenoma Radiation therapy takes prolong

period for effect (1‑10 years), require medical therapy for

intervening period and radiation induced hypopituitarism

which will affect fertility Hence on refusal for surgery she

was started on medical therapy Gamma knife therapy has

been used in the treatment of Cushing’s disease as primary

mode of therapy and after failed surgery.[3] During prolong

course of illness with two failed surgery, it becomes difficult

to persuade patient to undergo any intervention But we

could persuade patient to undergo gamma‑knife surgery

once it became available She responded with development

of adrenal insufficiency after 9 months Several studies have

been published in the use of gamma knife in functioning and

non‑functioning pituitary microadenomas Remission rate

defined by normalized 24hrs urinary cortisol levels ranged

from 35 to 83% of cases.[3‑6] The efficacy of remission

depended on the target volume[4] and the pre‑radiosurgery

hormonal levels.[5] Lower target volume and low hormonal

levels indicated higher the chances of remission The time

to remission varied from 13 to 24 months Our patient

went into adrenal insufficiency nine months post‑Gamma

knife Hypopituitarism is the most frequent side effect

frequently seen 12‑24 months post‑gamma knife decreasing

after about 120 months.[6] She needs regular follow up for

hypopituitarism So here we had a case that underwent

TNTS, bilateral adrenalectomy, medical treatment and

Gamma knife and finally achieved remission of disease

14 years after the diagnosis of Cushing’s disease suggesting

there is light at the end of the tunnel

M K Garg, Aditi Pandit, Sandeep Kharb

Department of Endocrinology, Army Hospital (Research and Referral), Delhi Cantonment, India

Corresponding Author: Dr M K Garg,

Department of Endocrinology, Army Hospital (Research and Referral), Delhi Cantonment ‑ 110 010, India

E‑mail: mkgargs@gmail.com

refereNCes

1 Young WF Jr, Thompson GB Laparoscopic adrenalectomy for

patients who have Cushing’s syndrome Endocrinol Metab Clin

North Am 2005;34:489‑99.

2 Newell‑Price J Diagnosis/differential diagnosis of Cushing’s

syndrome: A review of best practice Best Pract Res Clin Endocrinol

Metab 2009;23:S5‑14.

3 Kobayashi T, Kida Y, Mori Y Gamma knife radiosurgery in the

treatment of Cushing disease: Long‑term results J Neurosurg

2002;97:422‑8.

4 Jagannathan J, Sheehan JP, Pouratian N, Laws ER, Steiner L,

Vance ML Gamma Knife surgery for Cushing’s disease J Neurosurg

2007;106:980‑7.

5 Castinetti F, Nagai M, Morange I, Dufour H, Caron P, Chanson P, et al

Long‑term results of stereotactic radiosurgery in secretory pituitary

adenomas J Clin Endocrinol Metab 2009;94:3400‑7.

6 Castinetti F, Régis J, Dufour H, Brue T Role of stereotactic radiosurgery in the management of pituitary adenomas Nat Rev Endocrinol 2010;6:214‑23.

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Endocrinopathy complicating a case of Tuberous sclerosis

Sir, Tuberous sclerosis (TSC) is a multi‑system genetic disorder affecting skin, brain/nervous system, kidneys, heart, and lung and leading to growth of non‑malignant tumors A combination of symptoms may include seizures, developmental delay, behavioral problems, skin abnormalities, lung and kidney disease TSC is caused

by a mutation of either of two genes, TSC1 and TSC2, which code for the tumor suppressor proteins hamartin and tuberin, respectively

A 26‑year‑old female with no premorbidities presented with complaints of galactorrhea for the past 10 days and menstrual irregularities over the past 6 months Galactorrhea was spontaneous Her last childbirth 4 years ago was uneventful She had no headache, vomiting, visual impairment She denied any history of substance abuse, drug intake (anti‑psychotic, anti‑emetic, hormonal preparations), hypothyroidism, chronic liver or kidney diseases, epilepsy There was no significant family history She was a well‑nourished female with mild pallor, tiny nodule

on face, subungual fibroma in hands [Figures 1 and 2] There was spontaneous galactorrhea and mildly tender breasts without any signs of inflammation Systemic examination was entirely normal with normal IQ Ophthalmologic evaluation revealed white disk‑shaped retinal hamartoma Routine laboratory investigations including renal and liver function tests, thyroid profile were normal Serum prolactin was 85 ng/mL with FSH‑4.66 and LH‑4.21 mIU/ml

In view of the theoretical possibility of involvement of cell groups of other anterior pituitary lineage, tests for evaluation of other anterior pituitary hormones were carried out and were found to be normal Abdominal and pelvic

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