Diagnosis Of Pituitary Carcinoma Research Articles

THU108 Pituitary Carcinoma Diagnosed After Ten Years Of Smoldering Ectopic Nasopharyngeal Cushing’s

Abstract Disclosure: L. Carafone: None. E. Vates: None. M. Johnson: None. I. Shafiq: None. Introduction: Pituitary carcinomas are rare and typically carry a poor prognosis. Diagnosis of pituitary carcinoma is made when there is metastasis to one or more sites distant from the pituitary, including craniospinal or systemic metastases. Clinical Case: A 54-year-old man presented to the endocrine clinic in 2011 with recurrent urinary tract infections, thoracic compression fractures, uncontrolled hypertension with hypokalemia, and weight gain. Examination revealed facial plethora, supraclavicular fat pad, central obesity, multiple ecchymoses, proximal muscle weakness and muscle atrophy of the upper and lower extremities. Workup demonstrated abnormal 1 mg overnight dexamethasone suppression test (cortisol after 1 mg dexamethasone 25.7 mcg/dL, n≤1.8 mcg/dL), elevated salivary cortisol (4340 and 2950 ng/dL, n≤99 ng/dL), elevated 24 hr urinary-free cortisol (271.7 mcg/24hr, n≤60 mcg/24 hr), and elevated ACTH (240 pg/mL, n≤46 pg/mL). MRI brain showed a large, lobulated mass in the sphenoid sinus adjacent to the inferior left lateral aspect of the pituitary fossa. He underwent surgical resection of the mass and pathology was consistent with pituitary adenoma, corticotroph cell type. Following surgery, laboratory studies showed normal salivary cortisol (<50 ng/dL, n≤99 ng/dL) and 24 hr urinary-free cortisol (22.4 mcg/24hr, n≤60 mcg/24 hr). After two years of sustained remission, he experienced local recurrence for which he underwent repeat surgery with subsequent remission. One year later, this was again followed by recurrence for which he was treated with intensity-modulated radiation therapy and medical therapies including cabergoline, ketoconazole and eventually metyrapone. Six years after the second surgery, MRI showed increasing heterogeneous enhancement at the postoperative site, and he underwent a third surgery with tumor resection and the same pathology results as the initial resection. He was subsequently treated with fractionated proton therapy. He continued to require metyrapone to manage hypercortisolism despite reassuring serial MRI scans. A right neck mass was palpated in 2022 and CT demonstrated a right level 2b lymph node measuring 2.3 x 1.8 x 2.3 cm. FNA revealed neoplastic cells and he underwent right neck dissection with excision of the lymph node. Pathology was consistent with pituitary tumor, corticotroph cell type. Given metastasis of the pituitary tumor to a cervical lymph node, a diagnosis of pituitary carcinoma was made. Conclusion: The time course in pituitary carcinoma is highly variable from the initial diagnosis of the pituitary tumor to the diagnosis of metastatic disease. In this case, the diagnosis of pituitary carcinoma was made nearly one decade after the initial identification of a pituitary tumor. Metastatic disease should be considered in aggressive pituitary tumors when biochemical and radiologic findings are discordant. Presentation: Thursday, June 15, 2023

Pituitary carcinoma - case series and review of the literature.

Although pituitary adenomas (PAs) account for 15% of intracranial tumors, pituitary carcinomas (PCs) are a rare entity. Most commonly, PCs evolve from aggressive PAs invading the surrounding structures and eventually leading to metastatic lesions. Due to the low incidence, the diagnosis and treatment remains challenging. We report a case series of five patients with pituitary carcinoma (PC) treated in our center. At first diagnosis 3 patients had an ACTH-producing adenoma, 1 a prolactinoma and 1 a double secreting adenoma (GH and prolactin). The mean time interval from initial diagnosis to diagnosis of PC was 10.7 years (range 5-20 years). All patients underwent multiple surgical resections and radiotherapy. Four patients were treated with temozolomide for metastatic disease. One patient with concomitant radiochemotherapy for local recurrence. Temozolomide led to a stable disease in 2 patients. One patient had a progressive disease after 9 cycles of temozolomide. In absence of standard treatment, immunotherapy was initiated, resulting in a stable disease.We report five cases of PCs. Three patients obtained a stable disease after tailored multidisciplinary treatment. Additionally, one patient was treated with immunotherapy, opening a new treatment option in PCs. Overall, PCs are rare intracranial neoplasms occurring several years after the initial diagnosis of aggressive PAs. Currently, the absence of predictive factors for an aggressive clinical course, provokes a challenging management.

Liver metastases from pituitary carcinomas mimicking visceral well-differentiated neuroendocrine tumors: a series of four cases

BackgroundPathologists frequently encounter neuroendocrine tumors (NETs) presenting as multiple liver masses in routine practice. Most often, these are well-differentiated tumors with characteristic histologic features. In contrast, pituitary carcinoma is very rare, and there is limited data on its natural history and pathologic characterization.MethodsThe aim of this study was to describe clinical characteristics, histomorphology, immunophenotype and follow-up of pituitary carcinoma involving the liver and mimicking well-differentiated NETs of visceral origin. We selected a group of well-differentiated NETs of the pancreas to use as immunophenotypic controls. We identified 4 patients (age range, 51 to 73) with pituitary corticotroph carcinoma with liver metastases. Three patients presented with Cushing syndrome.ResultsAll cases histologically resembled well-differentiated NETs of visceral origin with Ki-67 proliferation indices of 5–42% and expression of T-PIT; metastatic tumors were not immunoreactive with CDX2, Islet 1 or TTF-1.ConclusionsFrequently, these cases display adrenocorticotropic hormone (ACTH) secretion and pituitary-specific transcription factor immunohistochemistry may be used as a reliable marker to distinguish metastatic pituitary carcinoma from NETs of visceral origin in addition to delineating a corticotroph carcinoma from somatotroph, lactotroph, thyrotroph, and gonadotroph lineage. Although rare, the differential diagnosis of pituitary carcinoma should be considered in metastatic well-differentiated NETs in which the site of origin is uncertain. In summary, pituitary corticotroph carcinoma can metastasize to the liver and mimic well-differentiated NET.

Pituitary carcinoma: The University of Texas MD Anderson Cancer Center experience.

2068 Background: Pituitary carcinoma (PC) is a rare and aggressive neuroendocrine tumor diagnosed once a pituitary adenoma (PA) becomes metastatic. Although no standard treatment currently exists, surgery, radiation (XRT) and/or chemotherapy are most commonly used. Recently, treatment with temozolomide (TMZ) has shown promising results, although the lack of prospective trials limits accurate outcome assessment. Methods: We describe a single-center multidisciplinary team experience in managing PC patients over a 13-year period (Oct, 2003 to Jan, 2017). Results: A total of 17 patients (9 males) were seen. Median age at diagnosis of PC was 44 years (range 16-82 years) and the median time from PA to PC conversion was 6 years (range 1-29 years). Median follow-up time: 28 months (range 8-158 months) with 7 reported deaths. The majority of PC was hormone-secreting (HS) subtype (n = 12): ACTH (n = 5), PRL (n = 4), FSH/LH (n = 2), GH (n = 1). After PC diagnosis, all pituitary tumors were locally invasive, all underwent at least one resection (mean 2.3) and all received at least one course of XRT alone (IMRT = 76%, SRS = 29%, IMPT = 12%) or concurrent with chemotherapy (18%). Immunohistochemistry showed high Ki-67 labeling index ( > 3%) in 9 (53%) cases. Most cases (n = 14) had metastases to the CNS, 35% of those had combined CNS and systemic foci. Ten (59%) cases underwent focal treatment of metastases, 90% of which underwent XRT. The most common chemotherapy used was TMZ [n = 14 (82%): single agent (n = 10); TMZ+capecitabine (n = 5); concurrent TMZ+XRT (n = 3); concurrent TMZ+XRT with adjuvant TMZ (n = 1)]. The median longest progression-free survival (LPFS) was 18 months (range 4-45 months). TMZ was associated with the chemotherapy-related LPFS in 9 (75%) cases. The 2, 3 and 5 year survival rate was 65%, 53% and 35%, respectively. All patients surviving > 5 years were treated with TMZ (n = 5, 2 survived > 10 years). Two refractory cases are currently on PD-1 inhibitor agent. Eighty percent of non-functioning PC were alive at last follow-up (HS-PC, 58%). Recurrence occurred in 11 (65%) cases. Conclusions: Treatment of PC requires a multidisciplinary approach. Multimodality therapy with surgery, radiation and TMZ was associated with higher survival rates and longer PFS.

Optic neuropathy following radiotherapy for Cushing's disease followed by the diagnosis of pituitary carcinoma

Optic neuropathy following radiotherapy for Cushing's disease followed by the diagnosis of pituitary carcinoma

Drop Metastasis of Adrenocorticotropic Hormone-Producing Pituitary Carcinoma to the Cauda Equina

The diagnosis of pituitary carcinoma cannot be made easily histologically, and most cases of pituitary carcinoma are diagnosed only after the clinical detection of metastasis. Distant metastasis of pituitary tumor occurs in 0.1% to 0.2% of cases and has been reported in the liver, bone and central nervous system, with only one case of metastasis to the cauda equine reported. This study describes a rare case of the drop metastasis of adrenocorticotropic hormone-producing pituitary adenocarcinoma to the cauda equina, causing cauda equina syndrome.

A case of prolactin-secreting pituitary carcinoma and its histological findings

Pituitary carcinomas are very rare. The diagnosis of pituitary carcinoma is defined by evidence of craniospinal and/or systemic metastasis, rather than by histological malignancies. We report a case of prolactin-secreting pituitary macroadenoma invading the cavernous sinuses at the time of initial treatment, which later metastasized to the cerebellum, medulla oblongata, and spinal axis. The patient survived approximately nine years following the initial diagnosis of a pituitary tumor and two years following the diagnosis of metastatic disease. Histological examination of the metastatic cerebellar tumor showed an adenoma with high cellularity and hyperchromasia, but no mitoses.

Pituitary Carcinomas.

Pituitary carcinomas are defined by their metastatic growth. Most of them also invade into surrounding tissues. They should be classified by the site of their metastases (cerebrospinal, systemic, or combined) and by the presumable cell type of origin, respectively with the hormone being demonstrable by immunohistochemistry (adrenocorticotrophic hormone [ACTH], prolactin [PRL], growth hormone [GH], hormone-negative). Pituitary carcinomas develop from invasive adenomas. Nearly all tumors had been treated by surgery or X-ray before they metastasized. Since 1976, 37 cases demonstrated with modern methods were reported: 23 had metastasized into the brain or meninges, 10 showed extracerebral metastases, and 4 showed both types of metastases. In our collection of pituitary tumors, three carcinomas (0.13%) were identified: two with systemic metastases (one ACTH secreting and one PRL secreting) and one with meningeal dissemination and ACTH production. The diagnosis of pituitary carcinomas should be based on four criteria: a demonstrable metastasis, identification of the primary tumor as a pituitary tumor, similarity between the structure and immunohistological marker expression of metastasis and primary tumor, and exclusion of an alternative primary tumor.