Transsphenoidal surgery (TSS) is first-line treatment for Cushing’s disease (CD), a devastating disorder of hypercortisolism resulting from overproduction of adrenocorticotropic hormone by a pituitary adenoma. Surgical success rates vary widely and disease may recur years after remission is achieved. Recognizing CD recurrence can be challenging; although there is general acceptance among endocrinologists that patients need lifelong follow-up, there are currently no standardized monitoring guidelines. To begin addressing this need we created a novel, systematic algorithm by integrating information from literature on relapse rates in surgically-treated CD patients and our own clinical experiences. Reported recurrence rates range from 3 to 47 % (mean time to recurrence 16–49 months), emphasizing the need for careful post-surgical patient monitoring. We recommend that patients with post-operative serum cortisol <2 µg/dL (measured 2–3 days post-surgery) be monitored semiannually for 3 years and annually thereafter. Patients with post-operative cortisol between 2 and 5 µg/dL may experience persistent or subclinical CD and should be evaluated every 2–3 months until biochemical control is achieved or additional treatment is initiated. Post-operative cortisol >5 µg/dL often signifies persistent disease and second-line treatment (e.g., immediate repeat pituitary surgery, radiotherapy, and/or medical therapy) may be considered. This follow-up algorithm aims to (a) enable early diagnosis and treatment of recurrent CD, thereby minimizing the detrimental effects of hypercortisolism, and (b) begin addressing the need for standardized guidelines for vigilant monitoring of CD patients treated by TSS, as demonstrated by the reported rates of recurrence.

Objective: Leiomyosarcomas are mesenchymal tumors that very rarely present as a primary adrenal malignancy. Methods: We present the complete clinical presentation, imaging, and anatomicopathologic features of a unique case of recurrent primary adrenal leiomyosarcoma (PAL) after initial surgery. This case is accompanied by an updated literature review. Results: A 63-year-old Hispanic female referred to our clinic 4 months after having right adrenalectomy. Eight months prior to this encounter, she started to notice bilateral lower extremity edema with skin changes. Computed tomography of the abdomen revealed a large 6.8 × 4.4 cm adrenal mass that appeared to be encroaching in the inferior vena cava with hypodense areas, irregular borders, and a necrotic center. A preoperative hormonal evaluation failed to reveal evidence of a functional adrenal tumor. Microscopic examination revealed spindle cell neoplasia. Immunohistochemistry of the surgical specimen revealed positive staining for desmin, smooth muscle actin, and focal myogenin (MYF-4). S-100 protein and inhibin were negative. The diagnosis of intermediate-grade leiomyosarcoma was confirmed. Two months after surgery, surveillance positron emission tomography scan revealed another right adrenal mass measuring 5.6 × 5 cm, with a standardized uptake value of 2.1. Conclusion: PAL is excessively rare. To our knowledge, this is only the second case of PAL with documented recurrence and the 19th case reported in the current medical literature. Surgical removal of the tumor is the first step in treatment. Adjuvant therapies are still not well standardized based on the low incidence of cases.

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