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Allergy & Rhinology (Providence, R.I.) 2020Craniopharyngiomas (CP) are suprasellar tumors that can grow into vital nearby structures and thus cause significant visual, endocrine, and hypothalamic dysfunction.... (Review)
Review
Craniopharyngiomas (CP) are suprasellar tumors that can grow into vital nearby structures and thus cause significant visual, endocrine, and hypothalamic dysfunction. Debate persists as to the optimal treatment strategy for these benign lesions, particularly with regards to the extent of surgical resection. The goals of tumor resection are to eliminate the compressive effect of the tumor on surrounding structures and minimize recurrence. It remains unclear whether a gross total resection (GTR) or subtotal resection (STR) with adjuvant therapy confers a better prognosis. Chemotherapy and radiation therapy (RT) have been explored as both neoadjuvant and adjuvant treatments to decrease tumor burden and prevent recurrence. The objective of this paper is to review the risks and benefits of GTR versus STR, specifically with regard to risk of recurrence and postoperative morbidity. Aggregated data suggest that STR monotherapy is associated with higher rates of recurrence relative to GTR (50.6% ± 22.1% vs 20.2% ± 13.5%), while STR combined with RT leads to recurrence rates similar to GTR. However, both GTR and RT are independently associated with higher rates of comorbidities including panhypopituitarism, diabetes insipidus, and visual deficits. The treatment strategy for CPs should ultimately be tailored to each patient's individual tumor characteristics, risk, symptoms, and therapeutic goals.
PubMed: 33240560
DOI: 10.1177/2152656720964158 -
Cureus Aug 2023The presence of pregnancy in a brain-dead woman is a rare circumstance. We present a case of a 31-year-old woman who was 22 weeks pregnant at the time of diagnosis of...
The presence of pregnancy in a brain-dead woman is a rare circumstance. We present a case of a 31-year-old woman who was 22 weeks pregnant at the time of diagnosis of brain death after intracranial and subarachnoid hemorrhage. After a multidisciplinary approach, the decision was made to continue somatic support to maintain the pregnancy until optimal fetus viability. Cesarean section was performed after 11 weeks (33 weeks gestational age) of brain-death diagnosis with a successful delivery of a live infant. Management of brain-death complications during pregnancy is described.
PubMed: 37753021
DOI: 10.7759/cureus.44172 -
Endocrinology, Diabetes & Metabolism... May 2023An 11-year-old girl with past medical history of septic shock and multi-organ failure at age 5 presented to her primary care doctor with concern for pallor of the lips....
SUMMARY
An 11-year-old girl with past medical history of septic shock and multi-organ failure at age 5 presented to her primary care doctor with concern for pallor of the lips. Laboratory studies demonstrated low free thyroxine (T4) and normal thyroid-stimulating hormone (TSH). A referral to endocrinology was made where the patient was evaluated, and laboratory evaluation was repeated. The patient was asymptomatic and clinically euthyroid with a height consistent with her mid-parental height and was in mid- to late-puberty. The repeated laboratory evaluation demonstrated a pattern suggestive of primary hypothyroidism with low free T4 and an elevated TSH. However, the magnitude of elevation of TSH was less than expected, given the degree of lowering of free T4; therefore, central hypothyroidism was considered. Workup was initiated, and laboratory studies and MRI imaging confirmed an underlying diagnosis of panhypopituitarism in the setting of pituitary stalk interruption syndrome.
LEARNING POINTS
Pituitary stalk interruption syndrome is a rare but important cause of panhypopituitarism. Central hypothyroidism should be suspected in patients with low free thyroxine with an inappropriate degree of elevation of thyroid-stimulating hormone. Workup of central hypothyroidism should include multi-pituitary hormone assessment, and, if evident, MRI imaging should be done. Adrenal insufficiency should be suspected in a hypotensive, critically ill patient who is failing to improve on standard-of-care therapy.
PubMed: 37183887
DOI: 10.1530/EDM-23-0021 -
Pituitary Jun 2024We aimed to investigate the prevalence and the diagnostic criteria of hypoprolactinemia in patients with panhypopituitarism and the effects of hypoprolactinemia on...
PURPOSE
We aimed to investigate the prevalence and the diagnostic criteria of hypoprolactinemia in patients with panhypopituitarism and the effects of hypoprolactinemia on depression and sexual functions.
MATERIALS AND METHODS
Forty-eight patients with panhypopituitarism and 20 healthy volunteers were included. Basal hormone levels were measured and a TRH stimulation test was performed. For the evaluation of sexual functions, questionnaries of Female Sexual Functional Index (FSFI) for females and International Erectile Functional Index for males were performed to the subjects. Depressive symptoms were evaluated by Beck Depression Envontory score (BDI-II).
RESULTS
The peak PRL response to TRH stimulation test at 5th percentile in the control group was 18.6 ng/ml in males and 41.6 ng/ml in females and accepted as the cut-offs for sufficient response of PRL. Prolactin was insufficient in 42(87.5%) patients. A basal PRL level of ≤ 5.7 ng/ml in males and 7.11 ng/ml in females was 100% specific in predicting an inadequate response to TRH stimulation test with 80% and 70% sensitivity respectively. A basal PRL level of ≥ 8.5 ng/dl in males was 100% specific and 76% sensitive, and in females a level of ≥ 15.2 ng/dl was 96% specific and 66% sensitive in predicting an adequate response to TRH. PRL deficient patients with panhypopituitarism had higher depression scores compared to the controls, lower sexual function scores in males.
CONCLUSION
PRL deficiency is prevalent among individuals with panhypopituitarism, with the potential to result in elevated depression scores in both sexes and impaired sexual functions in males. A basal PRL level seems to be sufficient for the diagnosis of hypoprolactinemia in routine clinical practice.
Topics: Humans; Male; Hypopituitarism; Female; Prolactin; Adult; Depression; Prevalence; Middle Aged; Thyrotropin-Releasing Hormone; Case-Control Studies; Young Adult
PubMed: 38700812
DOI: 10.1007/s11102-024-01393-0 -
BMJ Case Reports Jan 2021A man in his early 60s with a medical history of granulomatosis with polyangiitis (GPA) in remission for two decades without maintenance therapy presented with...
A man in his early 60s with a medical history of granulomatosis with polyangiitis (GPA) in remission for two decades without maintenance therapy presented with non-specific complaints of profound fatigue and 40-pound weight loss. He was seronegative for antinuclear antibodies and cytoplasmic antineutrophilic antibodies, but erythrocyte sedimentation rate and C reactive protein levels were elevated. Endocrinological testing revealed adrenal insufficiency, hypogonadism, hypothyroidism and diabetes insipidus. An MRI of the head revealed extensive sinonasal inflammation eroding through the floor of the sella turcica and into the pituitary gland and stalk. Biopsy of the sinonasal tissues was inconclusive. On review of his case, a multidisciplinary team diagnosed him with panhypopituitarism secondary to a recurrence of GPA. He responded well to glucocorticoids and methotrexate with marked reduction of pituitary enhancement on imaging and resolution of diabetes insipidus. He will require lifelong testosterone, levothyroxine and glucocorticoids for hormone replacement therapy.
Topics: Adrenal Insufficiency; Androgens; Diabetes Insipidus; Fatigue; Glucocorticoids; Granulomatosis with Polyangiitis; Hormone Replacement Therapy; Humans; Hypogonadism; Hypopituitarism; Hypothyroidism; Immunosuppressive Agents; Magnetic Resonance Imaging; Male; Methotrexate; Middle Aged; Pituitary Diseases; Pituitary Gland; Recurrence; Rhinitis; Sella Turcica; Sinusitis; Testosterone; Thyroxine; Weight Loss
PubMed: 33509867
DOI: 10.1136/bcr-2020-237774 -
Balkan Medical Journal Jul 2023
Topics: Humans; Autoimmune Hypophysitis; Hypopituitarism; Germinoma; Diagnostic Errors
PubMed: 37227236
DOI: 10.4274/balkanmedj.galenos.2023.2023-3-60 -
Surgical Neurology International 2021Malignant craniopharyngioma is a rare tumor with few published case reports. It can form or transform from a benign variant and is associated with a dismal survival... (Review)
Review
BACKGROUND
Malignant craniopharyngioma is a rare tumor with few published case reports. It can form or transform from a benign variant and is associated with a dismal survival rate. We reviewed the literature for all published cases and studied the effect of radiation on the rate of malignant transformation. We analyzed the effect of chemotherapy on survival.
METHODS
We used various search engines to locate literature from 1980 onward and identified 31 case reports, one of which was excluded. Statistical analysis using the SAS software was conducted, and a significant value was identified if < 0.05.
RESULTS
There was equal distribution among male and female patients. The average age at malignant diagnosis is 31.11 years (±15.16) and 12.19 years (±8.41) for the average interval of benign tumor progression to malignancy. The most common clinical presentation was visual loss and/or field deficits in 26/30 patients (86%). Almost 11/30 patients (37%) had endocrinological deficits, with panhypopituitarism as the most common in 8/11 patients (73%). Fifteen patients received radiation before malignant transformation (47%) and demonstrated no effect on malignant transformation ( = 0.379). Gross total resection was achieved in 2/30 patients. The average time to mortality postoperatively is 5.3 months ± 4.3. Ten patients received chemotherapy, and five were alive at last follow-up ( = 0.115).
CONCLUSION
Malignant craniopharyngioma carries a dismal prognosis with no apparent benefits of radiation therapy and chemotherapy on survival.
PubMed: 34754589
DOI: 10.25259/SNI_664_2021 -
Journal of Neurological Surgery. Part... Aug 2023Wide variations exist in the management of craniopharyngiomas, including pituitary stalk preservation/sacrifice. This study examines the practice patterns over 16...
Wide variations exist in the management of craniopharyngiomas, including pituitary stalk preservation/sacrifice. This study examines the practice patterns over 16 years using the endoscopic endonasal approach for the resection of craniopharyngiomas and it examines the effects of stalk preservation. Retrospective analysis was conducted for 66 patients who underwent endoscopic transsphenoidal surgery for resection of craniopharyngiomas. Patients were stratified into three epochs: 2005 to 2009 ( = 20), 2010 to 2015 ( = 23), and 2016 to 2020 ( = 20), to examine the evolution of surgical outcomes. Subgroup analysis between stalk preservation/stalk sacrifice was conducted for rate of gross total resection, anterior pituitary function preservation, and development of new permanent diabetes insipidus. Gross total resection rates across the first, second, and third epochs were 20, 65, and 52%, respectively ( = 0.042). Stalk preservation across epochs were 100, 5.9, and 52.6% ( = 0.0001). New permanent diabetes insipidus did not significantly change across epochs (37.5, 68.4, 71.4%; = 0.078). Preservation of normal endocrine function across epochs was 25, 0, and 23.8%; ( = 0.001). Postoperative cerebrospinal fluid (CSF) leaks significantly decreased over time (40, 4.5, and 0%; [ = 0.0001]). Stalk preservation group retained higher normal endocrine function (40.9 vs. 0%; = 0.001) and less normal-preoperative to postoperative panhypopituitarism (18.4 vs. 56%; = 0.001). Stalk sacrifice group achieved higher GTR (70.8 vs. 28%, = 0.005). At last follow-up, there was no difference in recurrence/progression rates between the two groups. There is a continuous evolution in the management of craniopharyngiomas. Gross total resection, higher rates of pituitary stalk and hormonal preservation, and low rates of postoperative CSF leak can be achieved with increased surgical experience.
PubMed: 37405242
DOI: 10.1055/s-0042-1751291 -
Medical Research Archives Oct 2022Snakebite is a preventable yet often-neglected public health hazard with high chronic disability and mortality, mainly faced by rural communities in the...
BACKGROUND
Snakebite is a preventable yet often-neglected public health hazard with high chronic disability and mortality, mainly faced by rural communities in the tropics/subtropics. Endocrinological disorders following snakebite (especially Russell's viper in India) are notably underrecognized and can lead to remarkable morbidity, poor quality of life, and cardiovascular mortality. Anterior pituitary insufficiency has been the most common ailment following Russell's viper envenomation amid those endocrinological dysfunctions. On the contrary, the posterior pituitary and nearby hypothalamus mostly remain unharmed, so central diabetes insipidus is extremely rare following a viperid snakebite envenomation.
CASE PRESENTATION
The authors present a patient developing panhypopituitarism with evident spontaneous central diabetes insipidus 29 years after Russell's viper envenomation. Relevant investigations ruled out other possible etiologies, and he responded well to hormonal replacement therapy.
CONCLUSIONS
Panhypopituitarism with concurrent central diabetes insipidus may occur following snakebite (especially in Russell's viper envenomation). Early recognition and proper management of these complications are quintessential to preventing further misdiagnosis, under-recognition, morbidity, impaired quality of life, and mortality.
PubMed: 36382205
DOI: 10.18103/mra.v10i10.3195 -
Neurology India 2022Despite the evolution of endoscopic techniques, large pituitary adenomas with unfavorable characteristics and irregular anatomical configurations continue to pose a...
BACKGROUND
Despite the evolution of endoscopic techniques, large pituitary adenomas with unfavorable characteristics and irregular anatomical configurations continue to pose a challenge for the dexterity, skills, and patience of endoscopic surgeons. Transcranial surgery retains a significant role in these situations where the tumor access, hemostasis, and dissection around adjoining neurovascular tissues can be controlled efficaciously.
OBJECTIVE
In this report, we describe our experience with transcranial surgery for pituitary adenomas highlighting its safety and versatility in peripheral centers.
METHODS
We accessed the case files and imaging records of pituitary tumors operated between 2001 and 2019 at a private hospital in a major Indian city. The records were analyzed with emphasis on postoperative clinical course, visual, and endocrinological outcomes. The data was analyzed with respect to differences between transcranial and transsphenoidal procedures. Categorical variables were compared with Chi-square test/Fischer's exact test and difference in means evaluated with Welch's t-test.
RESULTS
A total of 178 procedures were performed in 173 patients with pituitary adenoma, who were the subjects of this study. Ninety-eight (56.7%) patients were treated by transsphenoidal excision whereas 80 (46.2%) underwent transcranial procedures (75 primary and five secondary). In the patients operated transcranially, we observed three deaths and nine patients suffered from significant morbidity. Visual outcomes were similar to the group operated transsphenoidally. However, incidence of panhypopituitarism was significantly higher in transcranial procedures; the extent of resection was poorer than transsphenoidal surgeries owing to more extensive nature of tumors.
CONCLUSIONS
In low-volume centers, the endoscopic skills required for transsphenoidal resection of large and complex pituitary adenomas may be scarce. Transcranial surgery, dependent on familiar microsurgical techniques and equipment, may still be viable, safe, and an effective option.
Topics: Humans; Pituitary Neoplasms; Neurosurgery; Sphenoid Bone; Microsurgery; Treatment Outcome; Retrospective Studies; Adenoma
PubMed: 36352606
DOI: 10.4103/0028-3886.359173