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Abstract

Pituitary gland is an uncommon site of a primary cancer. Of more than 600 cases of pituitary tumors seen at the KFSH&RC between 1975 to 1998 only 3 patients had primary pituitary cancer. We have previously reported a case of pituitary fibrosarcoma arising as a rare complication of external radiotherapy (ERT) for GH-secreting pituitary adenoma (PA) [1]. We report now 2 cases of ACTH-producing primary pituitary carcinoma (ACTH-PPC); their follow-up data provide information on the natural history of this cancer. Patient #1; a 46 year old lady with Cushing's disease (CD) presented with an enlarged right cervical lymph node (LN) 2 years after having undergone a partial hypophysectomy through transsphenoidal surgery (PHYPX/TSS) and ERT for an invasive pituitary tumor. Patient #2; a 26 year old man presented with CD and underwent bilateral adrenalectomy (ADx) and pituitary ERT. Thirty-nine months later he developed Nelson's syndrome and a PHYPX/TSS was performed. Incidentally discovered hepatic metastases in this patient and an excisional biopsy of the LN in patient #1 showed histological features very similar to the pituitary tumor, and they stained strongly positive for ACTH. Perinuclear spherical hyalinized cytoplasmic inclusions were seen in the LN biopsy that corresponded to bundles of type 1 microfilaments (specific for pituitary ACTH-producing cells) seen by electron microscopy. A whole body 18-Fluoro-2-Deoxy-D-Glucose positron emission (FDG-PET) scanning, showed an intense uptake in the neck mass. A trial of octreotide did not change the exceedingly high levels of ACTH in patient #2, further supporting the diagnosis of ACTH-PPC. The clinical course of 102 months prior to his demise showed continued progression of the primary and the metastatic tumor. Patient #1, is alive at 15 months follow-up; hypercortisolemia is controlled using ketoconazole. ACTH-PPC should be entertained in a patient with CD presenting with persistent cervical lymphadenopathy. The clinical course in our patients suggests that the emergence of PC may involve a proliferative continuum from a pre-existing PA to an invasive tumor, culminating in a carcinoma. Adjunctive events such as ERT/ADx may predispose to the evolution of PC in genetically susceptible individuals. Because ERT is an effective treatment for PA its use will continue; it is important to be aware of the possible complication of primary pituitary carcinoma.

Orbital metastasis of pituitary growth hormone secreting carcinoma causing lateral gaze palsy

“…A total of 45 of 46 (97.8%) published cases described utilizing radiation therapy prior to metastatic progression were reviewed. [1–58–14161719–2325–272931–3739414445474850515658–6062] Eight patients had undergone stereotactic radiosurgery (Mean: 1.13 treatments) prior to progression. Forty patients underwent external radiation therapy (Mean: 1.11 treatments).…”

“…[58131719253951] Because of the close proximity of the cavernous sinuses and critical neurovascular structures, subtotal resection with follow up radiation therapy is a fairly common clinical scenario. [11213203158] Conventional radiotherapy is extensively utilized and yields excellent local rates of control. [30545557] Increasingly, radiosurgery has begun to gain favor as a modality producing similar control rates with possibly earlier secretory control, especially for GH-secreting adenomas.…”

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]).…”

“…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].…”