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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 -
Diagnostic Cytopathology Apr 2020Pituicytoma is a rare tumor of the sella and suprasellar region. It is common for these tumors to be misdiagnosed radiographically as pituitary adenomas, meningiomas,... (Clinical Trial)
Clinical Trial
BACKGROUND
Pituicytoma is a rare tumor of the sella and suprasellar region. It is common for these tumors to be misdiagnosed radiographically as pituitary adenomas, meningiomas, and craniopharyngiomas. Histologically, pituicytomas are also known to show variable morphology. These factors often complicate intraoperative consultation, especially when tissue is limited.
METHODS
A database search (January 1990-June 2019) identified 13 surgically resected pituicytomas that were sent for intraoperative consultation. The intraoperative cytology was reviewed by two pathologists, and both squash preparations/smears and touch preparations were included.
RESULTS
The cytological features of pituicytomas were variable. The cytoplasm ranged from fibrillary to fine and wispy. The nuclei were round to ovoid with occasional tumors showing spindled morphology. Small nucleoli were seen in all tumors, and chromatin was fine to vesicular. While squash preparations were cellular and revealed cohesive groups of tumor cells, touch preparations were often paucicellular with the exception of one tumor. The cytological features of pituicytomas are compared to those of other tumors with similar radiographic appearances.
CONCLUSIONS
Our findings illustrate the range of cytological features of these rare tumors and highlight the difficulty in making a definitive intraoperative diagnosis. Pituicytomas should be considered in the differential of a sellar/suprasellar lesion with glial and meningioma-like cytological features.
Topics: Adult; Aged; Female; Humans; Intraoperative Care; Male; Middle Aged; Pituitary Neoplasms
PubMed: 31883319
DOI: 10.1002/dc.24372 -
World Neurosurgery Apr 2020Pituicytoma is a rare neoplasm arising in the sellar region (World Health Organization grade I). Clinically, pituicytomas mimic nonfunctioning pituitary macroadenomas...
BACKGROUND
Pituicytoma is a rare neoplasm arising in the sellar region (World Health Organization grade I). Clinically, pituicytomas mimic nonfunctioning pituitary macroadenomas and are occasionally incidentally discovered at autopsy. Pituitary adenomas can occur with other sellar pathologies, and the term "collision sellar lesions" has been coined for this rare entity. There have only been a few reports of the coexistence of pituicytoma and pituitary adenoma. We present 2 cases of pituicytoma coexisting with acromegaly and Cushing disease.
CASE DESCRIPTION
Case 1: A 29-year-old woman had acromegaly. The macroadenoma was partially removed in her first surgery; thus an endonasal reoperation was required for debulking and posterior radiosurgery. Pituicytoma coexisting with somatotropinoma was diagnosed on pathologic examination. Case 2: A 33-year-old woman had adrenocorticotropic hormone-dependent Cushing disease. She underwent endonasal resection. Undetectable postoperative cortisol levels provided evidence that the underlying adrenocorticotropic hormone source was successfully removed. On the basis of morphologic features and the immunohistochemical profile, pituicytoma was diagnosed on pathologic examination. Pituitary adenoma was not confirmed histologically in this patient.
CONCLUSIONS
Only 117 cases of pituicytoma have been reported since it was first described in 1955. Before our report, only 5 cases of patients with pituicytoma coexisting with pituitary adenoma had been described. The coexistence of these 2 entities may not just be a mere coincidence but may be due to a yet unknown pathophysiologic link or common progenitor lineage of both lesions. Association between pituicytoma and pituitary adenoma is increasingly being reported.
Topics: Adult; Female; Growth Hormone-Secreting Pituitary Adenoma; Humans; Magnetic Resonance Imaging; Neoplasms, Multiple Primary; Pituitary ACTH Hypersecretion; Pituitary Gland, Posterior; Pituitary Neoplasms
PubMed: 31874293
DOI: 10.1016/j.wneu.2019.12.085 -
La Radiologia Medica Mar 2020Hypophysitis (HP) is a rare acute or chronic inflammatory condition of the pituitary gland. The greatest challenge in the management of HP is establishing a diagnosis... (Review)
Review
Hypophysitis (HP) is a rare acute or chronic inflammatory condition of the pituitary gland. The greatest challenge in the management of HP is establishing a diagnosis through clinical criteria and non-invasive methods and predicting the patients' clinical outcome. The aim of this review is to describe the neuroradiological findings of this rare disease, providing some information regarding the possible differential diagnosis in order to avoid unnecessary surgery. Gadolinium-enhanced pituitary magnetic resonance imaging (MRI) is considered the neuroradiological investigation of choice. The features suggestive for HP include an enlarged triangular- or dumbbell-shaped gland with a thickened and not obviously deviated stalk, further supported by the absence of posterior pituitary bright spot on T1weighted images, particularly in patients presenting with diabetes insipidus. Contrast enhancement pattern is quite variable; dural enhancement has been reported in some cases after intravenous contrast administration. The characterization of the unusual sellar mass is not straightforward and generally results in a wide differential. HP should be primarily differentiated from pituitary adenomas (including pituitary apoplexy), from pituitary metastases, and from other sellar and parasellar tumors, e.g., craniopharyngiomas, germinomas, gliomas, lymphomas, meningiomas, pituicytomas, chordomas, teratomas, dermoids and epidermoids, Rathke's cleft cysts, and abscesses. In patients suspected for secondary forms related to systemic pathology, additional imaging is helpful in identifying other involved sites. Neuroradiologists need to know MRI appearance of this rare disease, as well as its typical symptoms and serological markers. A strict collaboration with endocrinologists and neurosurgeons is mandatory in order to reach a definitive diagnosis, allowing to promptly initiating an appropriate treatment.
Topics: Adenoma; Autoimmune Hypophysitis; Contrast Media; Diagnosis, Differential; Gadolinium; Humans; Hypophysitis; Immunoglobulin G4-Related Disease; Magnetic Resonance Imaging; Neuroradiography; Pituitary Gland; Pituitary Neoplasms; Xanthomatosis
PubMed: 31863360
DOI: 10.1007/s11547-019-01120-x -
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 -
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 Feb 2020To evaluate and understand the clinical behavior and radiologic correlates of tumors originating from the posterior pituitary gland. To review the management strategy... (Review)
Review
OBJECTIVE
To evaluate and understand the clinical behavior and radiologic correlates of tumors originating from the posterior pituitary gland. To review the management strategy for these rare tumors and add to the limited existing literature.
METHODS
Retrospective review of 8 cases (5 pituicytomas, 2 spindle cell oncocytomas, and 1 granular cell tumor) managed at our institution between 2004 and 2019. The patients' clinical course, histologic features, and radiologic findings were reviewed. Their management and long-term follow-up is presented and compared with the literature.
RESULTS
Long-term follow-up ranged from 1 to 9 years. There was 1 recurrence in a patient with spindle cell oncocytoma, and this was treated with radiotherapy. The endoscopically managed cases resulted in complete tumor excision with no recurrence.
CONCLUSIONS
Epidemiologic data on primary tumors of the neurohypophysis is limited because of the rarity of these tumors. This study adds to the literature that these tumors behave as World Health Organization grade I tumors, although close follow-up is recommended as a few cases have shown recurrence. The endoscopic approach resulted in better gross total tumor resection rate in this series.
Topics: Adenoma, Oxyphilic; Adult; Aged; Aged, 80 and over; Cerebral Intraventricular Hemorrhage; Cytoreduction Surgical Procedures; Female; Glioma; Granular Cell Tumor; Hemianopsia; Humans; Hypogonadism; Incidental Findings; Male; Microsurgery; Middle Aged; Neoplasm, Residual; Neuroendoscopy; Pituitary Gland, Posterior; Pituitary Neoplasms; Sphenoid Bone
PubMed: 31734425
DOI: 10.1016/j.wneu.2019.11.043 -
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