Table 4.
Surgical and pathological findings and follow-up of patients with multiple endocrine neoplasia type 1 and Cushing’s syndrome.
| Surgery or Autopsy Findings |
Response to surgery and/or follow-up |
|||||
|---|---|---|---|---|---|---|
| Patient ID | Type of Operation/ Interventio n |
Operative Findings or Gross Pathology |
Pathology Results |
Earliest time UFCs or 17OHCS documented returned to normal post- op (days) |
Follow-up (months) |
Post-surgical or post-evaluation clinical course |
| Cushing's Disease Group | ||||||
| 1 | TSS | 2 separate adenomas: 11 mm left sided adenoma; 9 mm right adenoma. The right one invaded the cavernous sinus. | Left-sided tumor pos. PRL Right-sided tumor pos. ACTH | 4 | 4 | Clincally cured post-TSS. |
| 2 | TSS | No tumor found | No tumor found | 4 | 85 | Clinically cured post-TSS with blind left hemi-hypophysectomy; pt grew 12 cm and proceeded into puberty within 1 year. |
| 3 | TSS | 2 separate adenomas: Tumor 1= 3mm left superior adenoma; Tumor 2= 2 mm midline inferior adenoma | Tumor 1 pos. PRL, neg. ACTH Tumor 2 pos. PRL, neg. ACTH | 4 | 1 | Clincally cured post-TSS. |
| 4 | TSS | 3 mm right inferior adenoma | NA | 7 | 8 | Developed recurrent hypercortisolism within 5 months post-TSS at an outside institution; declined pituitary XRT and instead had bilat ADX |
| 5 | Bilat ADX, then transfrontal hypophys. and XRT | NA | NA | NA | 192 | Six years after bilat ADX, developed Nelson's syndrome, requiring transfrontal hypophys. and XRT; large residual pituitary mass evident up to 16 years later |
| 6 | TSS | Cystic/ necrotic adenoma invading left cavernous sinus | Tumor pos. for PRL, neg. for ACTH; probable nematode, toxoplasma cyst seen | 5 | 144 | Clincally cured post-TSS. |
| 7 | TSS | 3 mm left paramedial microadenoma | Pituitary microadenoma, Pos. PRL, neg. ACTH | 11 | 192 | Clincally cured post-TSS. |
| 8 | TSS | No tumor found | No tumor found | 11 | 264 | Clinically cured post-TSS with left hemi-hypophysectomy. |
| 9 | TSS | 5 mm right adenoma | Positive for ACTH | 5 | 48 | Clincally cured post-TSS. |
| 10 | TSS | 2 separate adenomas: Tumor 1= 3mm left inferior adenoma; Tumor 2= 5 mm right lateral adenoma, invading right cavernous sinus | Tumor 1 pos. PRL, GH Tumor 2 pos. ACTH Invading right cavernous sinus. | 4 | 96 | Clincally cured post-TSS. |
| 11 | TSS | Left microadenoma | Positive for ACTH | NA | 72 | Clinically cured post-TSS; lost 70 lbs. post-op |
| ACTH-independent Cushing's, Adrenal Tumor Group | ||||||
| 12 | Right ADX | Right adrenal tumor, 6 X 5 cm | adrenal cortical carcinoma, low-grade, with unequivocal vascular invasion | 120 | 108 | Post-op UFCs, serum cortisol nml within 4 months |
| 13 | TSS; later right ADX | no tumor found on TSS; 4 X 4 X 3 cm right adrenal adenoma | no tumor found on TSS; right adrenal adenoma found on ADX | 8 (following ADX) | 216 | Had failed TSS; became eucortisolemic after right adrenal tumor excised |
| 14 | Autopsy | 13 X 9.5 X 9 cm left adrenal mass pulmonary metasases |
left adrenal cortical carcinoma pulmonary metasases of adrenocortical cancer |
NA | NA | Died from complications of Cushings prior to surgical intervention; autopsy showed large left adrenal carcinoma metastatic to lung |
| Cushing's Syndrome from Unknown Etiology Group | ||||||
| 15 | Bilat ADX | Left adrenocortical tumor Right pheochromocytoma | NA (a) | NA (b) | 336 | Cured after bilat ADX and removal of left adrenal tumor and right pheochromocytoma; did not develop Nelson's syndrome over next 25 years |
| 16 | TSS | no tumor found | no tumor found | 420 | 228 | Persistent hypercortisolism for >4 months post-TSS; then spontaneous resolution sometime during next 10 months |
| 17 | NA (c ) | NA (c ) | NA (c ) | NA (c ) | NA (c ) | Hypercortisolism resolved spontaneously; never had required surgery. |
| 18 | NA (c ) | NA (c ) | NA (c ) | NA (c ) | 132 (since spont. resolution of CS) | Spontaneous resolution of hypercortisolism prior to scheduled TSS. |
| 19 | TSS | No tumor visualized; blind right hemi-hypophys. | 1 mm microadenoma; positive for ACTH | NA | 24 | Persistent hypercortisolism for at least 2 years post-TSS |
(a)
surgical pathology analysis performed at outside institution in 1971, prior to NIH evaluation, and not available.
(b)
postoperative biochemical data collected at outside institution in 1971, prior to NIH evaluation, and not available.
(c)
surgery to treat Cushing’s syndrome not performed.
Abbreviations: TSS, transsphenoidal pituitary surgery; PRL, prolactin; ACTH, adrenocorticotropic hormone; GH, growth hormone; CS, Cushing’s syndrome; UFC, urinary free cortisol; ADX, adrenalectomy; hypophys., hypophysectomy; Bilat., bilateral; NA, result or data not available.