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Endocrine Regulations Oct 2019Pituicytomas are rare, solid, well-circumscribed, low grade (grade I), non-neuroendocrine, and noninfiltrative tumors of the neurohypophysis or infundibulum, which...
OBJECTIVES
Pituicytomas are rare, solid, well-circumscribed, low grade (grade I), non-neuroendocrine, and noninfiltrative tumors of the neurohypophysis or infundibulum, which appear in the sellar/suprasellar regions. Herein, we present a case with Cushing's disease (CD) caused by an ACTH-secreting pituitary adenoma in association with an infundibular pituicytoma. Subject and Results. A 37-year-old male patient presented to the hospital with a six-month history of blurry vision. Physical examination demonstrated plethora, excessive sweating, weight gain, moon facies, and acne. Basal serum cortisol and ACTH levels were 16 µg/dl and 32 pg/ml, respectively. The results of screening tests were suggestive of Cushing syndrome. It was also 1.97 µg/dl following 8 mg dexamethasone suppression test which was consistent with CD. Pituitary MR imaging revealed a single lesion measuring 6x6.5 mm on the pituitary stalk. Infundibular mass excision and pituitary exploration by extended endoscopic endonasal approach were applied. On immunohistochemistry, strong diffuse immunolabeling for both S100 and TTF-1 was noted for the cells of infundibular mass, diagnosed as pituicytoma. Because the developed panhypopituitarism postoperatively, patient was discharged with daily desmopressin, levothyroxine, hydrocortisone, and intramuscular testosterone, once a month.
CONCLUSIONS
Pituicytoma is an uncommon noninvasive tumor of the sellar and suprasellar regions. In this case report, we described a patient with Cushing's disease to whom MRI displayed only an infundibular well-circumscribed lesion, but not any pituitary adenoma. Despite the absence of any sellar lesion, awareness of other undetected possible lesion and exploring hypophysis during the transsphenoidal surgery is mandatory for the correct diagnosis.
Topics: ACTH-Secreting Pituitary Adenoma; Adenoma; Adrenocorticotropic Hormone; Adult; Humans; Male; Neoplasms, Multiple Primary; Pituitary ACTH Hypersecretion; Pituitary Gland; Pituitary Neoplasms
PubMed: 31734654
DOI: 10.2478/enr-2019-0026 -
World Neurosurgery Apr 2020The majority of patients with acute lymphoblastic leukaemia develop disease relapse in the central nervous system in the absence of central nervous system-directed...
BACKGROUND
The majority of patients with acute lymphoblastic leukaemia develop disease relapse in the central nervous system in the absence of central nervous system-directed prophylactic therapy. In the past, prophylactic cranial irradiation was commonly used in the form of whole-brain radiotherapy in patients with acute lymphoblastic leukemia to prevent the development of intracranial diseases. However, in addition to the inherent risk of toxicity, this type of therapy has several delayed side effects including the development of secondary intracranial tumors.
CASE DESCRIPTION
We report a rare case of a patient with concurrent pituicytoma, meningioma, and cavernomas 44 years after prophylactic cranial irradiation for childhood acute lymphoblastic leukemia. The patient presented with visual disturbance, headache, and features of hypopituitarism. Endoscopic transsphenoidal resection of the pituicytoma and meningioma was performed. Subsequent regrowth of the residual meningioma necessitated further surgery and adjuvant treatment with radiotherapy.
CONCLUSIONS
This case report highlights the unusual case of a patient with 3 concurrent intracranial lesions of distinct pathologies after prophylactic cranial irradiation therapy for childhood acute lymphoblastic leukemia.
Topics: Adult; Female; Humans; Meningeal Neoplasms; Meningioma; Neoplasms, Radiation-Induced; Pituitary Neoplasms; Precursor Cell Lymphoblastic Leukemia-Lymphoma
PubMed: 31904435
DOI: 10.1016/j.wneu.2019.12.144 -
Zhonghua Bing Li Xue Za Zhi = Chinese... Jun 2022
Topics: Craniopharyngioma; Glioma; Humans; Magnetic Resonance Imaging; Pituitary Neoplasms
PubMed: 35673727
DOI: 10.3760/cma.j.cn112151-20220125-00055 -
International Journal of Surgery Case... Nov 2023Granular cell tumor (GCT) originating from the sellar and suprasellar regions, specifically from the neurohypophysis, is a rare neoplasm. Distinguishing GCT from other...
INTRODUCTION AND IMPORTANCE
Granular cell tumor (GCT) originating from the sellar and suprasellar regions, specifically from the neurohypophysis, is a rare neoplasm. Distinguishing GCT from other pituitary tumors, including pituitary adenoma, pituicytoma, and spindle cell oncocytoma, poses significant challenges. Here, we present a rare case of GCT originating from the posterior pituitary in the supra-sellar region.
CASE PRESENTATION
A 41-year-old woman, with no past medical history, presented to neurology department with decreased visual acuity and peripheral facial paralysis since 3 months. The MRI showed a well-defined supra-sellar, retrochiasmatic, oblong, hypothalamic expansive process. It was isointense T1-weighted, discretely hypotensive T2-weighted, measuring 19x17x16 mm, suggesting pituicytoma or craniopharyngioma. An endoscopic transsphenoidal surgical resection was performed. Microscopic examination showed a proliferation of diffuse architecture made up of rounded polyhedral cells with granular eosinophilic cytoplasm. On immunohistochemistry, tumor cells expressed diffusely TTF1, S-100 protein and SOX-10 confirming the diagnosis of supra-sellar GCT.
DISCUSSION AND CONCLUSION
GCTs are rare neoplasms that predominantly exhibit benign behavior, while the malignancy rate remains at 2 %. Histopathology serves as the definitive diagnostic approach for GCTs. These tumors are resistant to radiotherapy and chemotherapy, necessitating surgical resection as the primary treatment modality. Due to the potential absence of distinct tumor masses and local tissue infiltration by tumor cells, complete excision is crucial, with resection extent extending beyond areas of infiltration.
PubMed: 37883878
DOI: 10.1016/j.ijscr.2023.108977 -
Journal of Nuclear Medicine : Official... May 2024Small functional pituitary tumors can cause severely disabling symptoms and early death. The gold standard diagnostic approach includes laboratory tests and MRI, with or...
Small functional pituitary tumors can cause severely disabling symptoms and early death. The gold standard diagnostic approach includes laboratory tests and MRI, with or without inferior petrosal sinus sampling (IPSS). In up to 40% of patients, however, the source of excess hormone production remains unidentified or uncertain. This excludes patients from surgical, Gamma Knife, and CyberKnife therapy and adversely affects overall cure rates. We here assess the diagnostic yield of -(2-[F]fluoroethyl)-l-tyrosine ([F]FET) PET/MRI for detection of small functional pituitary tumors in these patients. This retrospective analysis included patients with Cushing disease (CD) but prior negative or inconclusive MRI results who underwent [F]FET PET/MRI between February 1, 2021, and December 1, 2022. PET/MR images and MR images alone were evaluated by experienced nuclear radiologists, neuroradiologists, or radiologists. Postoperative tissue analysis (when performed) was used as a reference standard to assess diagnostic metrics (i.e., sensitivity and positive predictive value). Results were also compared with previously obtained MR images, preceding IPSS, and clinical or biochemical follow-up. Twenty-two patients (68% female; mean age ± SD, 48 ± 15 y; range, 24-68 y) were scanned. All patients showed a clear metabolic focus on [F]FET PET, whereas reading of the MRI alone yielded a suspected lesion in only 50%. Fifteen patients underwent surgery directed at the [F]FET-positive focus. Tissue analysis confirmed a pituitary adenoma/pituitary neuroendocrine tumor of the corticotroph cell type (TPIT lineage) in 10 of 15 and a pituicytoma in 1 of 15, rendering a sensitivity of 100% and a positive predictive value of 73%. Lateralization was more accurate with [F]FET PET/MRI than with IPSS in 33%. Twelve of 16 (75%) patients who received surgical, Gamma Knife, or CyberKnife therapy after [F]FET PET/MRI reached short-term remission. [F]FET PET/MRI shows a high diagnostic yield for localizing small functional pituitary tumors. This multimodal imaging technique provides a welcome improvement for diagnosis, planning of surgery, and clinical outcome in patients with Cushing disease, particularly those with repeated negative or inconclusive MRI results with or without IPSS.
Topics: Humans; Female; Male; Middle Aged; Pituitary Neoplasms; Magnetic Resonance Imaging; Adult; Retrospective Studies; Positron-Emission Tomography; Tyrosine; Multimodal Imaging; Aged; Young Adult
PubMed: 38514085
DOI: 10.2967/jnumed.123.266853 -
Applied Immunohistochemistry &... Jan 2020Low-grade epithelial tumor of pituitary region with dominant papillary architecture is extremely rare. We describe a case of 20-year female who had a recurrent...
Low-grade epithelial tumor of pituitary region with dominant papillary architecture is extremely rare. We describe a case of 20-year female who had a recurrent nonfunctioning pituitary tumor. Histologic examination revealed a low-grade epithelial tumor with predominant papillary architecture, lined by cuboidal to columnar epithelial cells. The tumor cells were immunpositive for cytokeratin (CK), CK7, epithelial membrane antigen, carcinoembryonic antigen and showed diffuse and strong nuclear positivity for thyroid transcription factor 1. They were negative for neuroendocrine markers and pituitary hormones. Ki-67 proliferation index was low (1%). Ultrastructural examination revealed presence of microvilli, intercellular tight junctions, and keratin filaments within the tumor cells and lack of neurosecretory granules. No lesion was identified in thyroid or lung on systemic evaluation. On the basis of the morphology, immunophenotype, ultrastructural findings, and diffuse thyroid transcription factor 1 positivity, this tumor may represent an epithelial variant of pituicytoma with dominant papillary architecture. This type of differentiation is extremely rare, and to the best of our knowledge, has not been described previously in the literature.
Topics: Adult; Biomarkers, Tumor; DNA-Binding Proteins; Female; Humans; Neoplasm Proteins; Pituitary Hormones; Pituitary Neoplasms; Transcription Factors
PubMed: 31809313
DOI: 10.1097/PAI.0000000000000532 -
World Journal of Surgical Oncology May 2020Pituicytoma is a rare pituitary non-neuroendocrine tumour. The awareness of pituitary non-neuroendocrine tumours has gradually increased over the past several decades,...
BACKGROUND
Pituicytoma is a rare pituitary non-neuroendocrine tumour. The awareness of pituitary non-neuroendocrine tumours has gradually increased over the past several decades, but the knowledge of some histological variants of the tumours is limited, particularly in clinicopathological significance. Here, we report a rare case of pituicytoma variant.
CASE PRESENTATION
A 71-year-old man presented with sudden symptoms of stroke including urinary incontinence, weakness in right lower limb, and trouble speaking. Physical examinations showed a right facial paralysis. The radiological examinations eventually found a 1.7 × 1.4 × 1.3 cm sellar occupied lesion. After symptomatic treatment improved the symptoms, the patient underwent transsphenoidal resection of the pituitary mass. Histologically, the tumour contained hypocellular area and hypercellular area. The hypocellular area showed elongated spindle cells arranged in a fascicular pattern around small vessels and scattered Herring bodies; the hypercellular area showed a large number of pseudorosettes. Immunohistochemistrically, the tumour cells were positive for thyroid transcription factor-1, S100, and neuron-specific enolase. Neurofilament only showed a little positive in the hypocellular area, and silver impregnation was only noted in a perivascular distribution. The patient had no recurrence 4 months after the surgery.
CONCLUSIONS
The rare variant of pituicytoma has a favourable prognosis. Moreover, it needs to be distinguished pituicytomas with pseudorosettes from ependymomas because of different prognosis. Lastly, Herring bodies may occasionally be seen in the pituicytoma, which could be a potential diagnostic pitfall.
Topics: Aged; Biomarkers, Tumor; Humans; Magnetic Resonance Imaging; Male; Pituitary Gland, Posterior; Pituitary Neoplasms
PubMed: 32460843
DOI: 10.1186/s12957-020-01889-6 -
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 -
Neuropathology : Official Journal of... Dec 2023Granular cell tumors of the neurohypophysis (GCT) are rare benign neoplasms belonging, along with pituicytoma and spindle cell oncocytoma, to the family of TTF1-positive...
Granular cell tumors of the neurohypophysis (GCT) are rare benign neoplasms belonging, along with pituicytoma and spindle cell oncocytoma, to the family of TTF1-positive low-grade neoplasms of the posterior pituitary gland. GCT usually present as a solid sellar mass, slowly growing and causing compressive symptoms over time, occasionally with suprasellar extension. They comprise polygonal monomorphous cells with abundant granular cytoplasm, which is ultrastructurally filled with lysosomes. Here we report the case of a GCT presenting as a third ventricle mass, radiologically mimicking chordoid glioma, with aberrant expression of GFAP and Annexin-A, which lends itself as an example of an integrated diagnostic approach to sellar/suprasellar and third ventricle masses.
Topics: Humans; Pituitary Gland, Posterior; Third Ventricle; Granular Cell Tumor; Cerebral Ventricle Neoplasms; Craniopharyngioma; Pituitary Neoplasms; Glioma
PubMed: 37147874
DOI: 10.1111/neup.12907 -
Neurosurgical Focus Dec 2023The aim of this study was to report the authors' experience developing a Lean Six Sigma clinical care pathway (CCP) for endoscopic endonasal transsphenoidal operations.
OBJECTIVE
The aim of this study was to report the authors' experience developing a Lean Six Sigma clinical care pathway (CCP) for endoscopic endonasal transsphenoidal operations.
METHODS
Using Lean Six Sigma quality improvement principles-including the define, measure, analyze, improve, and control framework-the authors developed a CCP for endoscopic endonasal transsphenoidal operations, incorporating preoperative, intraoperative, and inpatient and outpatient postoperative phases of care. Efficacy and quality metrics were defined as postoperative length of stay (LOS), presentation to the emergency department (ED) or readmission within 30 days of discharge, and hospital charges. The study included all adult patients who underwent elective endoscopic endonasal resection for pituitary adenoma, Rathke's cleft cyst, craniopharyngioma, pituicytoma, or arachnoid cyst during the sampling period (April 1, 2018, to December 31, 2022).
RESULTS
Two hundred twenty-eight patients met criteria and were included; 94 were treated before and 134 were treated after implementation of the CCP. Differences between groups in age, gender, race, BMI, American Society of Anesthesiologists classification, geographic distribution, preoperative serum sodium, tumor size, adenoma functional status, and prior surgery were not significant. The mean postoperative LOS significantly decreased from 4.5 to 1.7 days following CCP implementation (p < 0.0001); LOS variability also decreased, with the standard deviation declining from 3.1 to 1.5 days. The proportion of patients discharged on postoperative day (POD) 1 significantly increased from 0% to 61.9% (p < 0.0001). Fewer than one-quarter of the patients (23.4%) were discharged by POD 2 prior to the CCP, while 88.8% of were discharged by POD 2 after CCP implementation (p < 0.0001). Rates of 30-day ED presentations or readmissions were not significantly different (2.1% vs 6.0%, p = 0.20, and 7.5% vs 6.7%, p > 0.99, respectively). Mean per-patient hospital costs declined from $38,326 to $26,289 (p < 0.0001), with an associated change in cost variability from a standard deviation of $16,716 to $12,498.
CONCLUSIONS
CCP implementation significantly improved LOS and costs of endoscopic endonasal resection, without adversely impacting postoperative ED presentations or readmissions.
Topics: Adult; Humans; Quality Improvement; Pituitary Neoplasms; Pituitary Gland; Nose; Endoscopy; Adenoma; Craniopharyngioma; Retrospective Studies; Treatment Outcome; Postoperative Complications
PubMed: 38039538
DOI: 10.3171/2023.9.FOCUS23545