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Praxis Jan 2024A 27-year-old man presented due to unilateral leg pain. He had a history of diabetes insipidus and panhypopituitarism. Laboratory analysis revealed hormonal undersupply....
A 27-year-old man presented due to unilateral leg pain. He had a history of diabetes insipidus and panhypopituitarism. Laboratory analysis revealed hormonal undersupply. MRI showed a large contrast medium-absorbing mass in the pituitary gland extending into the hypothalamus. FDG-PET/CT examination revealed a hypermetabolic soft tissue lesion around the left femoral shaft. After biopsy of the lesion, a diagnosis of multisystemic Langerhans cell histiocytosis was made.
Topics: Male; Humans; Adult; Positron Emission Tomography Computed Tomography; Hypopituitarism; Diabetes Insipidus; Pain; Diabetes Mellitus
PubMed: 38381107
DOI: No ID Found -
Acta Neurochirurgica Feb 2024Pituitary adenomas (PAs) usually have a soft consistency, facilitating gross total resection. However, 5-13% of PAs with fibrous consistency are challenging to remove...
PURPOSE
Pituitary adenomas (PAs) usually have a soft consistency, facilitating gross total resection. However, 5-13% of PAs with fibrous consistency are challenging to remove entirely and are accompanied by greater morbimortality. This study aims to identify the clinical and radiological characteristics that correlate with PA fibrous consistency preoperatively. A simple scoring system has been proposed to predict incidence of fibrous PAs.
MATERIALS AND METHODS
Consecutive interventions (226) were analyzed, all performed through an endoscopic endonasal transsphenoidal approach. Univariable and multivariable logistic regression analysis was performed. Hosmer-Lemeshow test and receiver operating characteristic (ROC) curves were assessed to evaluate the model. A point scoring system (PiTCon) was derived based on the multivariable regression model. Our study aimed to identify the clinical and radiological characteristics that correlate with fibrous tumor consistency preoperatively.
RESULTS
The best diagnostic accuracy for predicting PA consistency consisted of five predictive factors: age, compressive symptoms, panhypopituitarism, craniocaudal extension of the PA in mm, and prior surgery. The multivariable model achieved good discrimination with an area under the curve (AUC) of the ROC curve being 0.82 and the 95% CI 0.76 to 0.88. Internal validation yielded an optimism-adjusted C-statistic of 0.80 (95% CI 0.74 to 0.86). A point scoring system (PiTCon score) was designed using the best predictive model.
CONCLUSIONS
PA consistency can be estimated preoperatively regarding clinical and radiological characteristics. We propose a point-based scoring system (PiTCon score) that can better guide neurosurgeons in clinical decision-making and surgical risk assessment and help establish and describe patient prognosis.
Topics: Humans; Pituitary Neoplasms; Endoscopy; Hypopituitarism; Adenoma; ROC Curve; Retrospective Studies
PubMed: 38355813
DOI: 10.1007/s00701-024-05976-5 -
Clinical Case Reports Feb 2024Sheehan's syndrome may present with postpartum lactation failure and amenorrhea or with features of isolated hypopituitarism to panhypopituitarism. A high index of...
Sheehan's syndrome may present with postpartum lactation failure and amenorrhea or with features of isolated hypopituitarism to panhypopituitarism. A high index of suspicion is required in a relevant clinical setting of postpartum hemorrhage.
PubMed: 38344342
DOI: 10.1002/ccr3.8521 -
BMC Endocrine Disorders Feb 2024Lymphocytic hypophysitis is a rare autoimmune condition that usually presents during pregnancy and causes inflammation of the pituitary gland. Although the...
BACKGROUND
Lymphocytic hypophysitis is a rare autoimmune condition that usually presents during pregnancy and causes inflammation of the pituitary gland. Although the pathophysiology is not well understood, it often presents with headaches, visual disturbances, and symptoms of hypopituitarism. However, not all cases may present with hypopituitarism which can make this rare disease with an incidence of ~ 1 in 9 million much more difficult to diagnose.
CASE PRESENTATION
We present a 35-year-old G4P4 woman with progressive vision loss and intermittent frontal headaches during her first trimester through 2 months postpartum. She presented with no symptoms of hypopituitarism and her hormone panel only showed elevated prolactin, possibly due to her breastfeeding. She was treated with a right pterional craniotomy with decompression of both optic nerves, partial resection of the suprasellar mass, and glucocorticoid therapy for headaches and visual disturbances.
CONCLUSION
This case is notable for a presentation of lymphocytic hypophysitis without symptoms of hypopituitarism. This is important for outpatient providers to be aware of, especially those that care for pregnant patients so that unfavorable outcomes can be avoided.
Topics: Humans; Pregnancy; Female; Adult; Pituitary Neoplasms; Autoimmune Hypophysitis; Pituitary Diseases; Hypopituitarism; Pituitary Hormones; Headache; Magnetic Resonance Imaging
PubMed: 38326790
DOI: 10.1186/s12902-024-01546-z -
AACE Clinical Case Reports 2024Pituitary abscess is an uncommon life-threatening disease that could lead to panhypopituitarism. It is important to suspect its prevalence in regions with endemic...
BACKGROUND/OBJECTIVE
Pituitary abscess is an uncommon life-threatening disease that could lead to panhypopituitarism. It is important to suspect its prevalence in regions with endemic infectious diseases.
CASE REPORT
A 55-year-old man, a farmer, with a background of consumption of unpasteurized dairy products, presented with headache, impaired consciousness, and fever that started in February 2023. Initial test results were consistent with neuroinfection. Brain MRI showed ventriculitis; the pituitary gland was heterogeneous with the presence of an 8 × 8 mm abscess. The pituitary hormone axis was evaluated, and it showed results compatible with the results of panhypopituitarism with central hypothyroidism, central hypocortisolism, central hypogonadism, and growth hormone deficiency. Hormone replacement treatment with hydrocortisone and levothyroxine was started. The Rose Bengal test for and 2-mercaptoethanol Brucella agglutination test showed positive results. After neurobrucellosis (NB) was diagnosed, antibiotic treatment was commenced. The patient was discharged 6 weeks later and treatment with prednisone, levothyroxine, recombinant somatropin, testosterone, as well as doxycycline, and rifampin was continued for another 4 months.
DISCUSSION
NB and pituitary abscess are rare manifestations of brucellosis and are challenging to diagnose due to their nonspecific clinical presentation and cerebrospinal fluid (CSF) findings. NB diagnosis relies on neurologic symptoms and serological evidence of Brucella infection. Magnetic resonance imaging is the preferred diagnostic tool for pituitary abscesses. Medical management may be sufficient, while transsphenoidal drainage is not always necessary. Hormonal deficits typically remain permanent.
CONCLUSION
Pituitary abscess could be suspected in patients presenting with symptoms of neuroinfection, panhypopituitarism, and heterogenous image in the magnetic resonance imaging differential diagnosis. Opportune management can lead to reduced mortality and improved recovery of the pituitary hormone function.
PubMed: 38303770
DOI: 10.1016/j.aace.2023.10.005 -
Endocrine Journal Mar 2024Pituitary xanthogranulomatomas (XG) are a rare pathological entity caused by accumulation of lipid laden macrophages and reactive granuloma formation usually triggered...
Pituitary xanthogranulomatomas (XG) are a rare pathological entity caused by accumulation of lipid laden macrophages and reactive granuloma formation usually triggered by cystic fluid leakage or hemorrhage. Our aim was to compare clinical characteristics and presenting features of patients with secondary etiology of XG and those with no identifiable founding lesion (primary -"pure" XG) in order to gain new insights into this rare pituitary pathology. In a retrospective review of 714 patients operated for sellar masses, at tertiary center, we identified 16 (2.24%) with histologically confirmed diagnosis of pituitary XG over the period of 7 years (2015-2021). Patients were further analyzed according to XG etiology: "pure"- XG (n = 8) with no identifiable founding lesion were compared to those with histological elements of pituitary tumor or cyst - secondary XG (n = 8). We identified 16 patients (11 male), mean age 44.8 ± 22.3 years, diagnosed with pituitary XG. Secondary forms were associated with Ratke's cleft cyst (RCC, n = 2) and pituitary adenoma (PA, n = 6). The most common presenting features in both groups were hypopituitarism (75%), headache (68.5%) and visual disturbances (37.5%). Predominance of male sex was noted (males 68.75%, females 31.25%), especially in patients with primary forms. Patients with primary pituitary XG were all males (p = 0.0256) and more frequently affected by panhypopituitarism (87.5% vs. 25%, p = 0.0406) compared to patients with secondary causes. Hyperprolactinemia was noted in pituitary tumor group with secondary etiology only (p = 0.0769). Majority of lesions were solid on magnetic resonance imaging - MRI (81.25%). Distinct clinical phenotype was observed dependent on the etiology of XG.
Topics: Female; Humans; Male; Young Adult; Adult; Middle Aged; Aged; Pituitary Neoplasms; Pituitary Diseases; Pituitary Gland; Magnetic Resonance Imaging; Central Nervous System Cysts; Cysts; Granuloma; Xanthomatosis
PubMed: 38281757
DOI: 10.1507/endocrj.EJ23-0398 -
Pituitary Apr 2024To explore the potential role of focused radiotherapy in managing the lymphocytic hypophysitis (LH) refractory to medical therapy and surgery.
Successful treatment of medically and surgically refractory lymphocytic hypophysitis with fractionated stereotactic radiotherapy: a single-center experience and systematic literature review.
PURPOSE
To explore the potential role of focused radiotherapy in managing the lymphocytic hypophysitis (LH) refractory to medical therapy and surgery.
METHOD
A systematic literature review was conducted following PRISMA guidelines to identify the studies on radiation treatment for hypophysitis, along with the experience in our institution.
RESULTS
The study included eight patients, three from our institution and five from existing literature. The age at presentation ranged from 37 to 75 years old, with a median age of 58. The presenting symptoms involved headache in seven patients and diplopia in two patients. Pre-radiation visual field defects were noticed in four patients. All patients exhibited variable degrees of hypopituitarism before radiation, with oral corticosteroids being the initial medical treatment. Immunosuppressive therapy was attempted in two patients prior to radiation. Seven patients had a history of transsphenoidal surgery with a histologically confirmed LH. Three patients underwent stereotactic radiosurgery (SRS), while the remaining received FSRT, with a mean irradiation volume of 2.2 cm. A single-session total dose of 12 -15 Gy was administered in the SRS group. In the FSRT group, doses ranged from 24 to 30 Gy with a median dose of 25 Gy, delivered in 2 Gy fractions. Four patients achieved a resolution of visual field defects, while another two patients demonstrated improvement in their associated focal neurologic deficits. No change in pre-existing endocrine status was shown after radiation, except in one patient. Clinical response was achieved in seven patients after a single course of radiation, while one patient required the second course. Six patients remained stable on low-dose glucocorticoid during at least a 12-month follow-up period, and one discontinued it entirely without experiencing relapse. Three patients demonstrated a complete radiologic response, while the remaining showed a partial radiologic response.
CONCLUSIONS
Focused radiation, including FSRT, can play a role in symptomatic relief, effective mass shrinkage, and minimizing radiation exposure to critical surrounding structures in patients with refractory LH. However, further research efforts are necessary to better clarify its effects and optimal dose planning.
Topics: Humans; Adult; Middle Aged; Aged; Radiosurgery; Autoimmune Hypophysitis; Dose Fractionation, Radiation; Hypopituitarism; Treatment Outcome; Retrospective Studies
PubMed: 38270722
DOI: 10.1007/s11102-023-01367-8 -
Cureus Dec 2023Heterozygous mutations of ()can result in ocular malformations, pituitary abnormalities, or hypopituitarism spanning from isolated growth hormone (GH) deficiency to...
Heterozygous mutations of ()can result in ocular malformations, pituitary abnormalities, or hypopituitarism spanning from isolated growth hormone (GH) deficiency to combined pituitary hormone deficiency. We present a patient exhibiting growth and pubertal disturbances, developmental delay, and pigmentary retinopathy. Further examination revealed deficiencies in GH following clonidine stimulation, hypogonadism, and, subsequently, central hypothyroidism. Brain magnetic resonance imaging uncovered hypoplasia of the pituitary and an ectopic pituitary tissue. Sequence analysis of identified a novel heterozygous mutation c.555_556dup, p.(Ser186Ilefs*21), indicative of a frameshift mutation. Replacement therapy with recombinant human GH, testosterone enanthate, and levothyroxine was started. Notably, GH therapy resulted in significant catch-up growth. This case report contributes to our comprehension of the molecular and clinical findings, particularly highlighting endocrine manifestations and a rare ophthalmologic manifestation associated with mutations in the gene.
PubMed: 38249203
DOI: 10.7759/cureus.50819 -
Clinical Neurology and Neurosurgery Jan 2024Radiotherapy (RT) is the corner stone of nasopharyngeal carcinoma (NPC) treatment but it exposes to late effects especially hypothalamic pituitary deficiency (HPD). In...
PURPOSE
Radiotherapy (RT) is the corner stone of nasopharyngeal carcinoma (NPC) treatment but it exposes to late effects especially hypothalamic pituitary deficiency (HPD). In this article,we aimed to assess the impact of RT on pituitary function in NPC survivors.
METHODS
We included 55 patients treated in the radiation oncology department, of Farhat Hached Hospital in Sousse, Tunisia.
RESULTS
All patients received facio-cervical RT with a mean dose of 73.3 Gy to the nasopharynx. After a mean follow up of 9.56 years, 34 patients (61.8%) presented HPD. Associated peripheral involvement was seen in 18.2%. The most prevalent deficiency was of the GH axis in 50.9% followed by secondary adrenal insufficiency in 20%. Panhypopituitarism was noted in 8.8%. The development of HPD wasn't related to RT dose (OR: 0.41(0.05-2.92), p = 0.36) but was significantly associated with male gender (OR: 1.67 (1.21-2.37), p = 0.01).
CONCLUSION
HPD post RT is a common phenomenon. Therefore, we recommend regular assessment of pituitary function amongst patients treated with RT for NPC because identification of deficits is crucial to allow early and appropriate hormone replacement therapy in order to improve patients quality of life.
Topics: Humans; Male; Nasopharyngeal Carcinoma; Quality of Life; Hypothalamo-Hypophyseal System; Pituitary Gland; Pituitary Diseases; Nasopharyngeal Neoplasms
PubMed: 38218060
DOI: 10.1016/j.clineuro.2023.108100 -
Case Reports in Endocrinology 2023Parathyroid carcinoma accounts for <1% of cases of primary hyperparathyroidism (PHPT). This rare condition may present with severe hypercalcemia and bone complications...
BACKGROUND
Parathyroid carcinoma accounts for <1% of cases of primary hyperparathyroidism (PHPT). This rare condition may present with severe hypercalcemia and bone complications such as osteoclastomas and pathologic fractures. Here, we present a rare condition of panhypopituitarism resulting from an osteoclastoma in the sphenoid bone that invaded the pituitary fossa due to parathyroid carcinoma. . A 47-year-old woman previously diagnosed with PHPT underwent a parathyroidectomy 6 years earlier, with histological examination indicating a parathyroid adenoma. After surgery, she continued to exhibit high serum parathyroid hormone (PTH) and calcium levels, with the development of bone pain and spontaneous fractures. Imaging exams showed a large osteoclastoma of the sphenoid bone, invading the pituitary fossa, causing hypopituitarism. A new parathyroidectomy was performed, with histological confirmation of parathyroid carcinoma and regression of the osteoclastoma.
CONCLUSION
This case illustrates an unusual presentation of parathyroid carcinoma, in which an osteoclastoma of the sphenoid bone caused hypopituitarism.
PubMed: 38156081
DOI: 10.1155/2023/8274108