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HONEY, I BLEW UP THE KID! A SUPER Sized Medical Grand Rounds

HONEY, I BLEW UP THE KID! A SUPER Sized Medical Grand Rounds. Presentor: Suzette Grace R. Kho, M.D. Resource Persons: Eric Flores, M.D. (Neurosurgery) Paolo Villanueva, M.D (Pathology) Gerardo Beltran, M.D. (Radiology) Teresa Sy-Ortin, M.D. (Radio-oncology )

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HONEY, I BLEW UP THE KID! A SUPER Sized Medical Grand Rounds

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  1. HONEY, I BLEW UP THE KID! ASUPERSized Medical Grand Rounds Presentor: Suzette Grace R. Kho, M.D. Resource Persons: Eric Flores, M.D. (Neurosurgery) Paolo Villanueva, M.D (Pathology) Gerardo Beltran, M.D. (Radiology) Teresa Sy-Ortin, M.D. (Radio-oncology) Moderator: Thelma Crisostomo, M.D.

  2. To present a case of a 17 year old female with unusually tall stature To discuss differential diagnosis, & work-up for patients presenting with pituitary mass to discuss pathophysiology & treatment options for patients presenting with pituitary tumors Objectives

  3. Identifying Data F.O. • 17 Female • Filipino • Cagayan de Oro Chief Complaint: Evaluation of Tall Stature

  4. > 95th % 198 cm 191cm 185 cm 168 cm 154 cm 121 cm

  5. 3 mos PTA (+) “dimming” of her peripheral vision. (+) difficulty guarding her opponents, & would sometimes miss catching a pass. no consult was done until an annual school PE Tall stature Delayed development of secondary sexual characteristcs Visual field abnormalities CONSULT

  6. Review of Systems • (-) rashes, (-) skin pigmentation • (-) nocturia, (-) polyuria, (-) polydipsia • (-) palpitations, (-) tremors, (-) heat/ cold intolerance, (-)weight gain/ weight loss • (-) chest pain , (-)no difficulty of breathing, (-) lactation • (-) easy fatigability, (-) body weakness, (-) tetany, (-) muscle cramps • (-) hirsutism

  7. Developmental History • Delivered term via NSD to a 24 y/o G2P1 • Birth weight: 6 lbs. • Birth length: claims to be within normal • No delivery complications • unremarkable • unremarkable

  8. Family History • Father- 5’4” (162.54 cm) • Mother- 5’3” (160 cm), menarche at age 12 *midparental height: 154.77cm • 1 Sibling: Brother- 5’9” (175.26 cm) • tall relatives >182 cm ( 6’): mother side -8 uncles-: 6’ to 6’2” -1 male cousin: 6’1” • (+) thyroid disease- aunt • (+) HPN- father, uncle • (+) asthma- father and brother • (+) CVA- grandfather • (-) DM, (-) colon cancer

  9. Physical Examination • Conscious, coherent, ambulatory, oriented to 3 spheres • Vital Signs: BP 110/70 mmHg HR 84 bpm RR 20 cpm T 36.3°C

  10. Anthropometrics wt: 95.5kg ht: 198cm BMI: 24.1(overweight)   upper segment: 84.84 cm lower segment: 113cm U/L segment: 0.76 6’6”

  11. Anthropometrics ht: 198cm Arm span - 205.74 cm

  12. Physical Examination (+) depressed anterior (5.5 cm x 3.5cm) & posterior (1.5cm x1.0cm) fontanelles no coarsening of features (+) slightly thickened & widened nose and lips (+) gap between incisors, with slight prominence of jaw

  13. Physical Examination Lipomastia with no distinct glandular tissues External genitalia: female pubic hair, with no clitoral enlargement, bright pink vaginal mucosa, no milky secretions noted.

  14. Physical Examination (+) prominent hands and feet, with thickening of the soles of the foot Full & equal pulse

  15. Neurologic Examination Awake, alert, oriented to 3 spheres Pupils 3-4mm ERTL, EOM full and equal, (+) ROR, (+) visual field cuts Can smile, frown, clench teeth, tongue midline on protrusion Can shrug shoulders MMT: 5/5 on all extremities Sensory: 100% intact No dysmetria, no dysdiadokinesia (-) Brudzinski’s (-) Kernig’s (-) Babinski DTR: ++

  16. Salient Features Depressed fontanelles slightly thickened & widened nose and lips upper tooth gap with slight prominence of jaw Prepubertal Tanner stage 1 breasts Tanner stage 3 pubic hair Prominent hands and feet • 17 year old female • Tall stature • Delayed puberty • Headaches • Visual field defect

  17. Differentials

  18. Initial Impression Gigantism probably secondary to Growth hormone secreting pituitary adenoma with Hypogonadotrophic Hypogonadism

  19. Clinical Features of Gigantism/ Acromegaly Work Up IGF-1 Level Normal for Age & Gender Elevated Dx excluded Measure OGTT & GH levels Adequate GH suppression Inadeguate / no GH suppression Surgery Assess likelihood of surgical success Piuitary Mass Pituitary MRI Medical (Clinical features figure from Minkowski O. Ueber einen Fall von Akromegalie. Berliner Klinische Wochenschrift 1887;21:371-374; from Melmed S. Medical progress: acromegaly. N Engl J Med 2006;355:2558-2573. Erratum in N Engl J Med 2007;356:879).

  20. Work up NONSUPPRESSION

  21. MRI of Brain (+)pituitary mass Referred to a neurosurgeon Transsphenoidal Surgery

  22. GENETIC STUDY AUTOMATED PERIMETRY VISUAL FIELD TEST LEFT EYERIGHT EYE TEMPORAL TEMPORAL HEMIANOPSIA HEMIANOPSIA WITH CENTRAL SPARING

  23. Hormones

  24. Pelvic Ultrasound Infantile uterus, with thin endometrium (3mm) Small left ovary, right ovary not visualized

  25. Skeletal Survey

  26. Bone Aging

  27. Bone Mineral Density NORMAL SPINE FEMORAL NECK

  28. 2 D Echocardiogram Ultrasound of the Neck and Thyroid Gland

  29. Course in the Wards: • Dec. 2, 2010: transnasal transphenoidal resection of the pituitary mass • Post op: • (+) mild transient DI, treated with small doses Desmopressin 100 mcg/tablet • Hydrocortisone 100mg/IV was maintained and tapered in 4 days. Transient sugar elevations noted, given insulin injections. • 7th HD: discharged, on Prednisone 7.5mg/tab (5mg- 0-2.5mg).

  30. PATIENT: F.O. 17/FSPECIMEN NO.: 10-SR-0282

  31. IMMUNOHISTOCHEMICAL STAIN: GROWTH HORMONE, POSITIVE (>90%)

  32. IMMUNOHISTOCHEMICAL STAIN PROLACTIN, POSITIVE (<5%)

  33. Follow Up NONSUPPRESSION NONSUPPRESSION

  34. Follow Up

  35. Follow Up

  36. Follow Up Repeat MRI 3 mos. after surgery: mass slightly decreased but appears unchanged April 29, 2010: Repeat transsphenoidal surgery

  37. Updates

  38. Updates NONSUPPRESSION NONSUPPRESSION NONSUPPRESSION

  39. Updates

  40. CASE SUMMARY • MRI of the brain: (+) pituitary mass • Histopathological & Immunohistochemical Findings: GH & Prolactin Macroadenoma • Elevated • GH levels • IgF-1 level • Prolactin level • Prepubertal • Estradiol • FSH and LH level Infantile uterus Normal Karyotype- • 46 XX • 17 year old female • Tall stature • Prominently enlarged hands and feet • Slightly widened and thickened nose and lips • Widened upper tooth gap Delayed puberty Tanner stage 1 breasts tanner stage 3 pubic hair Bitemporal Hemianopsia

  41. Final Diagnosis Gigantism secondary to Growth Hormone and Prolactin Co- Secreting Pituitary Macroadenoma with stalk compression resulting in hypogonadotrophic hypogonadism, s/p Transnasal Transphenoidal Surgery (12/2/09 & 4/29/10)

  42. The Pituitary Gland & Mass Effects of Pituitary Tumor BITEMPORAL HEMIANOPSIA COMPRESSION OF PITUITARY STALK HEADACHES HYPERPROLACTINEMIA HYPOPITUITARISM

  43. Hypothalamic Pituitary Axis Hypothalamus Pituitary Trophic H. Target Organ

  44. In our patient.. HORMONE HYPERSECRETION Growth Hormone Secreting Adenoma (>90%) Prolactin Co-Secreting Adenoma (<5%) (Mammasomatotrophs) GIGANTISM Combined GH and Prolactin over-secretion common in early childhood gigantism Lafferty, A.R & Chrousos, G.P., Pituitary tumors in Children & Adolescents. J. Clin. Endccinol. Metab. 1999.84:4317-4323.

  45. Gigantism Identical twins, 22 years old, excess GH secretion

  46. Acromegaly

  47. Causes of GIGANTISM / Acromegaly >98% Melmed SM, ed. Acromegaly: A Comprehensive Guide to Diagnosis and Treatment. East Hanover, NJ:Novartis; 2003. Melmed S. Medical progress: acromegaly. N Engl J Med 2006;355:2558-2573. Erratum in N Engl J Med 2007;356:879. 49

  48. Clinical Features of Acromegaly Melmed S. N Engl J Med 2006;355:2558-2573

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