-
Medicine Nov 2019Pituicytomas are exceptional rare tumors in the sellar and suprasellar regions with clinical manifestations, such as headache, visual disturbance, hypopituitarism, and... (Review)
Review
RATIONALE
Pituicytomas are exceptional rare tumors in the sellar and suprasellar regions with clinical manifestations, such as headache, visual disturbance, hypopituitarism, and decreased libido. Unlike that of common pituitary adenoma, the association between pituicytoma and Cushing disease (CD) is extremely rare. There were only 6 reported cases till now. In the current study, we describe an unusual case of pituicytoma associated with severe CD with a recurrence-free follow-up period of 49 months.
PATIENT CONCERNS
A 32-year-old woman was referred to our hospital with moon face, central obesity, and purple stripes on the lower limbs.
DIAGNOSES
The plasma cortisol level was 1122 nmol/L. The low-dose dexamethasone suppression test failed to suppress plasma cortisol. This test provided evidence of nonpituitary-dependent CD. However, magnetic resonance imaging demonstrated a sellar mass measuring approximately 7.6 × 5.7 mm. The patient was diagnosed with pituitary microadenoma. Histopathological analysis of the tissue sections based on the findings from the immunohistochemical staining diagnosed it as pituicytoma.
INTERVENTIONS
Transsphenoidal surgery was performed to remove the pituitary mass.
OUTCOMES
Within 2 months postoperatively, the patient's blood pressure and cortisol level decreased gradually and normalized on the 6th month when other symptoms of CD also disappeared. The patient is presently free from recurrence 49 months after the initial diagnosis.
LESSONS
Based on the postoperative remission, CD was caused by pituitary disorders. A reasonable assumption is that an extremely small coexisting adenoma was not detected by the surgeon and washed out during the dissection. Another possible explanation might be the mass effect caused by this intrasellar lesion.
Topics: Adenoma; Adult; Female; Humans; Pituitary ACTH Hypersecretion; Pituitary Neoplasms
PubMed: 31689841
DOI: 10.1097/MD.0000000000017772 -
Journal of Clinical Medicine Oct 2019The hypothalamus is the site of synthesis and secretion of a number of endocrine peptides that are involved in the regulation of hormonal activity of the pituitary and... (Review)
Review
The hypothalamus is the site of synthesis and secretion of a number of endocrine peptides that are involved in the regulation of hormonal activity of the pituitary and other endocrine targets. Tumors of the hypothalamus have been recognized to have both structural and functional effects including hormone hypersecretion. The classification of these tumors has advanced over the last few years, and biomarkers are now available to classify these tumors and provide accurate structure-function correlations. This review provides an overview of tumors in this region that is critical to metabolic homeostasis with a focus on advances in the diagnosis of gangliocytomas, neurocytomas, and pituicytomas that are unique to this region.
PubMed: 31635149
DOI: 10.3390/jcm8101741 -
Pituitary Dec 2019This case series evaluates the surgical management of granular cell tumor (GCT) of the sellar region. This rare entity presents a unique diagnostic and surgical...
PURPOSE
This case series evaluates the surgical management of granular cell tumor (GCT) of the sellar region. This rare entity presents a unique diagnostic and surgical challenge.
METHODS
Institutional neuropathology databases at Brigham and Women's Hospital and Massachusetts General Hospital were searched for cases with a tissue diagnosis of GCT, and with a location in the sellar region. Patient, treatment, tumor, and follow-up data were extracted.
RESULTS
Three patients had a diagnosis of GCT of the sellar region occurring over an 18-year period. All three patients were followed postoperatively at our multidisciplinary pituitary center (median follow-up = 30 months; range 12-30 months). Hormonal disturbances, an incidental lesion requiring diagnosis, and neurological symptoms were indications for surgery in these patients. Two patients underwent a craniotomy and one underwent endoscopic transsphenoidal surgery. All three patients were free of tumor recurrence at last follow-up. In one case tested, positive thyroid transcription factor-1 (TTF-1) immunohistochemistry was observed.
CONCLUSION
GCT is generally a benign tumor of the sellar region. Surgical resection is the standard treatment, more recently with transsphenoidal surgery when indicated. Surgical resection results in optimal outcome for patients.
Topics: Adult; Female; Granular Cell Tumor; Humans; Male; Middle Aged; Neoplasm Recurrence, Local; Pituitary Diseases; Pituitary Neoplasms
PubMed: 31620953
DOI: 10.1007/s11102-019-00999-z -
Acta Neuropathologica Communications May 2019Pituicytoma is a rare, poorly characterized tumor of the sellar region that is thought to be derived from neurohypophyseal pituicytes. Resection of pituicytomas is often...
Pituicytoma is a rare, poorly characterized tumor of the sellar region that is thought to be derived from neurohypophyseal pituicytes. Resection of pituicytomas is often associated with significant morbidity including diabetes insipidus and panhypopituitarism. Most of the literature on this tumor exists as small case series or case reports. Here we describe a cohort of fourteen pituicytoma resections from eleven patients. The average follow-up on these cases is 3.7 years with some patients having over 10 years of follow-up data available in the electronic medical record. Pituicytomas were frequently misdiagnosed on pre-operative imaging, and surgical resection was associated with persistent endocrine abnormalities. Histologically, the tumors showed a range of morphologies from epithelioid to spindled. All tumors were positive for TTF-1 with variable immunostaining for other markers including GFAP, EMA, S100, SSTR2A, and synaptophysin. Within this cohort are two patients with atypical pituicytomas which showed increased cellularity, pleomorphism, mitoses and elevated Ki-67 proliferation indexes when compared to non-atypical pituicytomas. Next generation sequencing performed on three tumors revealed alterations in genes involved in the MAPK pathway. Additionally, immunohistochemical staining for phosphorylated-ERK was positive in the majority of tumors. Increased awareness of the neoplastic entity and identification of targetable mutations have the potential to decrease the morbidity associated with resection of pituicytomas.
Topics: Adult; Aged; Biomarkers, Tumor; Female; Humans; Immunohistochemistry; MAP Kinase Signaling System; Male; Middle Aged; Pituitary Neoplasms; Proto-Oncogene Proteins B-raf
PubMed: 31046843
DOI: 10.1186/s40478-019-0722-6 -
Neurology India 2018Spindle cell oncocytoma (SCO) is a newly described rare entity simulating clinicoradiological features of a nonfunctional pituitary adenoma and is corresponding to the... (Review)
Review
Spindle cell oncocytoma (SCO) is a newly described rare entity simulating clinicoradiological features of a nonfunctional pituitary adenoma and is corresponding to the category of World Health Organization grade I tumor. However, because of the reported incidence of recurrence and invasive presentation in some cases, its categorization as a low grade tumor is questionable. Earlier, it was thought to arise from the folliculostellate cells of adenohypophysis. Recently, few reports have described expression of thyroid transcription factor-1 [TTF-1], which is a specific marker for pituicytes of neurohypophysis, suggesting this tumor to be a variant of pituicytoma. We describe a case of SCO in a 28-year-old young female patient with TTF-1 immunopositivity, and ultra-structurally showing abundant mitochondria along with few neurosecretory granules.
Topics: Adenoma, Oxyphilic; Adult; Female; Humans; Pituitary Neoplasms; Thyroid Nuclear Factor 1
PubMed: 30233016
DOI: 10.4103/0028-3886.241353 -
Surgical Neurology International 2018Pituicytomas are considered World Health Organization Grade I malignancies. Until September 2017, a total of 81 cases of pituicytomas were diagnosed and described in...
BACKGROUND
Pituicytomas are considered World Health Organization Grade I malignancies. Until September 2017, a total of 81 cases of pituicytomas were diagnosed and described in literature. We present such a case in which histopathology shows an epithelioid pattern, a rare variant of pituicytoma. As far as we know, this is only the second such case described in the literature.
CASE DESCRIPTION
A 61-year-old male patient presented with complaints of progressive decrease in visual acuity for about 7 months, worse on the left side. Laboratory and endocrinological investigation returned normal values. Magnetic resonance imaging revealed a mixed solid-cystic lesion, measuring about 3.1 × 2.2 × 2.9 cm. The lesion presented with intermediate signal intensity in T1 and T2 sequences and showed avid postcontrast enhancement. The patient underwent resection through a left pterional approach. Pathology revealed a glial neoplasm with an epithelioid pattern and moderate cellularity with rounded-elongated cell nuclei and with a broad eosinophilic cytoplasm. Absence of cellular pleomorphism, any mitotic figures, or areas of necrosis was noted.
CONCLUSION
The epithelioid variant of pituicytomas differs from the commonly encountered forms of this tumor which typically present in a fascicular pattern. Microsurgical resection is the treatment of choice. However, in many cases, subtotal resection was performed because of a considerable risk for neurovascular injuries.
PubMed: 30105139
DOI: 10.4103/sni.sni_319_17 -
Oncology Letters Jul 2018Pituicytomas are a rare form of indolent neoplasms, which typically present with visual disturbance and hypopituitarism. Complete resection by a trans-sphenoidal...
Pituicytomas are a rare form of indolent neoplasms, which typically present with visual disturbance and hypopituitarism. Complete resection by a trans-sphenoidal approach is the optimal treatment. Only 80 cases have been described thus far in the current literature and the present case is the first to describe the development of pituitary apoplexy in the context of a pituicytoma. A 77-year-old man presented with fatigue and clinical signs of hypogonadism and a sellar lesion was diagnosed at cerebral magnetic resonance imaging (MRI). A watch-and-wait management was initially decided and 1 year after the initial diagnosis, he presented with a thunderclap headache with images suggestive of pituitary apoplexy. A pituitary adenoma was suspected and an endoscopic resection was decided upon the development of a visual deficit. Pathological analysis established the correct diagnosis of a pituicytoma. Pituicytomas are characterised by dense vascularisation, thus ischaemic and haemorrhagic events may be common. When confronted with a hypervascularised pituitary lesion demonstrating strong contrast enhancement and no abnormal hormonal secretion, one must maintain a high index of suspicion for a pituicytoma. A wide range of differential diagnoses should thus be considered in the context of pituitary apoplexy.
PubMed: 29928451
DOI: 10.3892/ol.2018.8625 -
Journal of Radiology Case Reports May 2017Hemangioblastomas are rare vascular tumors most often found in the posterior fossa and cervical spinal cord and commonly associated with von Hippel-Lindau Disease. We...
Hemangioblastomas are rare vascular tumors most often found in the posterior fossa and cervical spinal cord and commonly associated with von Hippel-Lindau Disease. We report a case of sporadic hemangioblastoma in a patient without von Hippel-Lindau Disease. Imaging characteristics included a solid, suprasellar mass that was homogeneously enhancing. These findings most resembled a pituicytoma or choroid glioma because of the close association with the infundibulum and the homogeneous avid enhancement. Microscopically, the neoplasm was seen to be composed of vascular channels associated with foamy stromal cells, containing clear cytoplasmic vacuoles. Microscopic and immunohistochemical findings were consistent with hemangioblastoma. Hemangioblastomas are a rare form of vascular tumor most commonly associated with von-Hippel Lindau disease. Our finding of non-cystic hemangioblastoma arising from the infundibulum demonstrates that, while rare, hemangioblastomas should be considered on the differential diagnosis for an avidly enhancing suprasellar mass.
Topics: Adult; Endoscopy; Female; Hemangioblastoma; Humans; Magnetic Resonance Imaging; Pituitary Gland; Pituitary Neoplasms
PubMed: 29299088
DOI: 10.3941/jrcr.v11i5.2981 -
Brain Tumor Research and Treatment Oct 2017A 19-year-old man presented with bitemporal hemianopsia and was found to have a large sellar and suprasellar tumor, resembling a pituitary macroadenoma. Emergency...
A 19-year-old man presented with bitemporal hemianopsia and was found to have a large sellar and suprasellar tumor, resembling a pituitary macroadenoma. Emergency transsphenoidal approach was attempted because of rapid visual deterioration with headache. However, the approach was complicated and stopped by uncontrolled hemorrhage from the tumor. After conventional cerebral angiography and recognition of an unusual pathology, transcranial approach was achieved to prevent permanent visual loss. The final pathological diagnosis was pituicytoma with epithelioid features. Pituicytoma is a rare low-grade tumor (WHO Grade I) of pituicytes involving the sellar and suprasellar region, and originating from special glial cells of the neurohypophysis. Because of the high vascularity, the firm consistency, and invasion to surrounding neurovascular structures, a pituicytoma should be included in the differential diagnosis of a mass in the sellar and suprasellar area if the tumor shows high enhancement with vascular components. We report a case of rare pituicytoma mimicking a pituitary macroadenoma with massive hemorrhage to disturb surgery.
PubMed: 29188213
DOI: 10.14791/btrt.2017.5.2.110 -
CNS Neuroscience & Therapeutics Jul 2017Pituicytoma is a rare neoplasm of the sella region. Tumor resection is the primary treatment option, but remains subtotal due to excessive bleeding in many cases. The...
INTRODUCTION
Pituicytoma is a rare neoplasm of the sella region. Tumor resection is the primary treatment option, but remains subtotal due to excessive bleeding in many cases. The search for alternative or additional treatment regimens is necessary.
AIMS
We aimed to determine the receptor expression of pituicytoma to find alternatives or supplements to surgical therapy in the use of targeted therapies.
METHODS
Pituicytoma samples were collected from three institutions between 2006 and 2015 and were stained for vascular endothelial growth factors (VEGF), thyroid transcription factor (TTF1), and somatostatin receptors (SSTR 2/3/5). The stains were classified from 0=no staining to +++=strong staining. A complementary retrospective analysis of the patient charts regarding sex, age, and primary symptoms, pituitary function, and perioperative complications was performed.
RESULTS
Ten samples were analyzed; mean patient age was 57.8 years SD 16.3 years. Seven samples were acquired from male patients (one relapse) and three from female. All tumors stained strongly positive (+++) for VEGF-R. Six samples stained positive for TTF1. As for somatostatin receptors, three samples were slightly positive for SSTR 2; seven were negative. SSTR 3 was + in one, three were ++, three were +++, and three were 0. SSTR 5 stained +++ in 1, ++ in 5, + in 1, and 0 in three patients.
CONCLUSIONS
Pituicytomas were generally positive for VEGFR and showed regular expression of SSTR 3 and 5 indicating a possible treatment option through targeted therapies in cases where resection remains insufficient. Further research is necessary as to whether tumor growth can be inhibited using these pathways.
Topics: Adult; Aged; DNA-Binding Proteins; Female; Humans; Intraoperative Complications; Male; Middle Aged; Neoplasm Recurrence, Local; Pituitary Neoplasms; Postoperative Complications; Receptors, Somatostatin; Retrospective Studies; Transcription Factors; Vascular Endothelial Growth Factor A; Young Adult
PubMed: 28556544
DOI: 10.1111/cns.12709