An ACTH-Producing Pituitary Carcinoma Developing Cushing's Disease

A Case of Corticotroph Carcinoma that Caused Multiple Cranial Nerve Palsies, Destructive Petrosal Bone Invasion, and Liver Metastasis

“…Of the 53 reported patients, including 34 women and 19 men, only 32 subjects were diagnosed as having Cushing' syndrome or disease at initial presentation [5–9, 12–18, 20–22, 27–32, 34, 35, 38, 39, 43]. The remaining 21 cases were endocrinologically asymptomatic, except for one case of galactorrhea–amenorrhea syndrome [25], and presented with the pituitary mass compressing the adjacent tissues [10, 11, 19, 23, 24, 26, 27, 33, 36, 37, 40–42, 44, 45]: visual disturbance in 13 cases, headache in one case, and visual impairment plus headache in five cases (symptoms not described in one case [39]). These 21 hormonally asymptomatic tumors, which were initially diagnosed as non-functioning adenomas, can be divided into two subgroups on the basis of the subsequent clinical course.…”

“…The most common is corticotroph carcinoma, representing 42% of pituitary carcinomas [1–4], and 53 cases of corticotroph carcinoma have been described in the English literature so far [5–45]. At the time of diagnosis, 32 (60%) of the 53 cases presented with symptoms and signs of chronic hypercortisolemia characteristic of Cushing's disease [5–9, 12–18, 20–22, 27–32, 34, 35, 38, 39, 43], while 15 (28%) of the 53 cases developed these features 6 months to 12 years after an initial presentation as a pituitary mass compressing the surrounding tissues [10, 11, 19, 23–26, 33, 36, 37, 39, 40, 42, 44, 45]. Although very rarely, silent corticotroph carcinoma has also been recognized by the presence of metastatic lesions in the absence of clinical features associated with chronic hypercortisolemia [27, 41].…”

Cytopathologic Features of Pituitary Carcinoma with Cervical Vertebral Bone Metastasis

“…5,15,16 No distinguishing histopathologic features between adenoma and carcinoma exist. 4,6 The recognition of craniospinal and distant metastasis is generally accepted as the true diagnostic hallmark of carcinoma. 1,5 Prevalence of pituitary carcinoma, based on a review of > 3,000 adenohypophysial tumors resected at the Mayo Clinic, was 0.2%.…”

“…1,3 Common distant metastatic sites are liver, cervical lymph nodes, bone and lung. [1][2][3][4][5][6]9,10,17 Only 39 cases of ACTHproducing pituitary carcinomas have been described in the literature to date. 8,10 Thirty-four of them were recently reviewed by Holthause et al, 18 and those authors noted that 16 (45%) cases exhibited extracranial …”

“…1,2,5 To the best of our knowledge, approximately 70 pituitary carcinomas have been reported in the literature. 1,[6][7][8][9][10] Although cytopathologic features of pituitary adenomas were well defined in squash smears, those of extracranial metastatic pituitary carcinomas were not. [11][12][13] Only 2 cases have been diagnosed correctly by fine needle aspiration biopsy so far.…”