Rathke Cleft Cysts with Apoplexy-Like Symptoms: Clinicoradiologic Comparisons with Pituitary Adenomas with Apoplexy

Jung, Hye Na; Kim, Sung Tae; Kong, Doo Sik; Suh, Sang Il; Ryoo, Inseon

doi:10.1016/j.wneu.2020.03.086

Cited by 7 publications

(4 citation statements)

References 31 publications

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“…Although a patient with RCC may present with apoplexy-like symptoms, there may not always be evidence of a hemorrhage. 19–21 The lack of hemorrhage, along with the presence of inflammatory cells, in our patient’s pathology argues against RCC hemorrhagic apoplexy as the underlying pathological process.…”

Section: Discussionmentioning

confidence: 72%

Rathke’s cleft cyst presenting with recurrent aseptic meningitis and inflammatory apoplexy: illustrative case

Murayi

Trivedi

Almeida

et al. 2023

Journal of Neurosurgery: Case Lessons

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BACKGROUND Rathke’s cleft cyst (RCC) is a benign sellar/suprasellar lesion often discovered incidentally. Rarely, symptomatic cases can present with headache and may exhibit concomitant aseptic meningitis or apoplexy. The authors describe a patient with an RCC presenting with recurring episodes of aseptic meningitis and ultimately inflammatory-type apoplexy. OBSERVATIONS A 30-year-old female presented with three episodes of intractable headaches over 2 months. Each episode’s clinical picture was consistent with meningitis though cerebrospinal fluid cultures, and viral tests remained negative. Imaging demonstrated a sellar lesion, initially thought to be coincidental. On the third presentation, there was rapid interval growth of the lesion, adjacent cerebritis, and new endocrinopathy. Resection was then performed via an endoscopic endonasal approach. Pathology showed an RCC with acute and chronic inflammation and no evidence of hemorrhage. Cultures were negative for organisms. The patient received several weeks of antibiotic treatment with the resolution of all symptoms and no recurrence. LESSONS Recurrent aseptic meningitis with apoplexy-like symptoms is a rare presentation of RCC. The authors propose the term inflammatory apoplexy to describe such a presentation without evidence of abscess, necrosis, or hemorrhage. The mechanism is unclear although may be due to intermittent microleakage of cyst contents into the subarachnoid space.

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Section: Discussionmentioning

confidence: 72%

Rathke’s cleft cyst presenting with recurrent aseptic meningitis and inflammatory apoplexy: illustrative case

Murayi

Trivedi

Almeida

et al. 2023

Journal of Neurosurgery: Case Lessons

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“…A novel GDF9:c.1364A>C stop-loss variant is reported in a Greek patient with secondary amenorrhea and a pituitary lesion compatible with either a Rathke cyst or a pituitary macroadenoma. Of note, Rathke cyst rupture and pituitary adenoma hemorrhage may present indistinguishably 9,10 and the patient did experience symptoms of severe pituitary apoplexy at ages 25 and 38. The hormonal profile (Supplemental Table 1, http:// links.lww.com/MENO/A878) including increased levels of the glycoprotein a-subunit could be consistent with a gonadotropin secreting adenoma, whereas recurrent elevation of FSH levels along with the presence of ovarian cysts and hyperestrogenemia may indicate ovarian hyperstimulation as reported for both FSH-secreting adenomas and perimenopause.…”

Section: Discussionmentioning

confidence: 89%

Ovarian insufficiency and secondary amenorrhea in a patient with a novel variant within GDF9 gene

et al. 2022

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Objective: Premature ovarian insufficiency is a heterogeneous condition that can be caused by several factors, such as genetic, environmental, etc. and represents one of the main causes of female infertility. One of the genes implicated is GDF9, which encodes a member of the transforming growth factor-beta superfamily that participates in the coordination of somatic cell activity, female fertility, including folliculogenesis, and oocyte maturation.Damaging variants in GDF9-encoded growth factors can cause the production of inhibin, perturb oocyte granulosa cell microenvironments, and obstruct follicle development. A novel GDF9 variant is herein reported to consolidate the role of GDF9 in ovarian function and female fertility.Methods: A 38-year-old female was referred for the investigation of secondary amenorrhea. Eventually, she was referred for genetic evaluation whereby conventional karyotyping and Fragile-X molecular testing were normal. Whole Exome Sequencing was performed, followed by targeted Sanger sequencing in all family members for variant confirmation and evaluation.Results: In this study we report a patient presenting with secondary amenorrhea due to premature ovarian failure and a pituitary lesion with radiological characteristics compatible with a Rathke cyst or a macroadenoma, residing between the adenohypophysis and neurohypophysis. Whole Exome Sequencing revealed a novel heterozygous stoploss variant c.1364A>C, p.(Ã455SerextÃ8) in the GDF9 gene.Conclusions: Should the predicted elongated GDF9 protein and differentially configurated GDF9 mature protein molecule form unstable dimers, rapid proteolytic degradation may take place and inhibit homo/heterodimer formation.

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“…It is also important to note that the radiological anomalies related to conservatively treated apoplexy usually show tumour shrinkage after several months ( 1 ) whereas haemorrhagic RCCs remain stable or even grow at imaging. Hints for the presence of RCC also include T1-hyperintensity related to protein content within the cyst ( 8 ) and the absence of nodular enhancement ( 9 ).…”

Section: Discussionmentioning

confidence: 99%

Conservative management of complicated Rathke’s cleft cyst mimicking pituitary apoplexy

Constantinescu

Wilms

Furnica

et al. 2022

Endocrinology, Diabetes &Amp; Metabolism Case Reports

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Summary Complicated Rathke’s cleft cyst (RCC) is a rare occurrence of symptomatic bleeding or growth of a previously asymptomatic (and often undiagnosed) intrasellar cyst derived from remnants of Rathke’s pouch, situated on the midline between the adeno- and neurohypophysis. Symptoms may be identical to those of pituitary apoplexy: acute onset of headache, hypopituitarism, and neurological disturbances. Both syndromes may also exhibit a similar appearance of a large haemorrhagic sellar mass at initial radiological evaluation. We report on two patients who presented with headache and complete hypopituitarism. Based on the initial MRI, they were first diagnosed with pituitary apoplexy but managed conservatively with hormone therapy alone because of the absence of severe visual or neurological threat. Upon follow-up at 4 months, clinical evolution was good in both patients but their pituitary mass had not reduced in size and, after careful radiologic reviewing, was more indicative of a large midline complicated RCC. In conclusion, the diagnosis of complicated RCC is challenging because it can mimic pituitary apoplexy clinically, biologically, and radiologically. Clinicians should distinguish between the two entities using specific radiological signs or evolution of the mass at MRI if the patient does not undergo surgery. To our knowledge, we report conservative management of this rare condition for the first time, though it seems appropriate in the absence of neurological compromise or visual compression. Long-term follow-up is however mandatory. Learning points Complicated Rathke’s cleft cyst can mimic pituitary apoplexy, presenting with sudden onset of headache, hypopituitarism, and visual and neurological compromise in the most severe cases. At diagnosis, pituitary MRI may not be able to differentiate between the two entities, showing a large haemorrhagic mass inside the sella, with little or no normal pituitary tissue visible. Patients are often diagnosed with apoplexy at this stage and may undergo pituitary surgery. When surgery has not been performed initially in these patients, repeat imaging at 3–6 months is unchanged and does not show the expected involution usually seen after adenoma apoplexy. Conservative management with hormonal replacement seems a valid option in the absence of visual or neurological deficits that would require trans-sphenoidal surgery.

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Rathke Cleft Cysts with Apoplexy-Like Symptoms: Clinicoradiologic Comparisons with Pituitary Adenomas with Apoplexy

Cited by 7 publications

References 31 publications

Rathke’s cleft cyst presenting with recurrent aseptic meningitis and inflammatory apoplexy: illustrative case

Rathke’s cleft cyst presenting with recurrent aseptic meningitis and inflammatory apoplexy: illustrative case

Ovarian insufficiency and secondary amenorrhea in a patient with a novel variant within GDF9 gene

Conservative management of complicated Rathke’s cleft cyst mimicking pituitary apoplexy

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