Reversible Cerebral Vasoconstriction Syndrome Associated with a Suprarenal Mass

Abstract

Reversible cerebral vasoconstriction syndrome (RCVS) is characterised by severe thunderclap headaches (with or without the presence of acute neurological symptoms) and segmental vasoconstriction of cerebral arteries that resolves spontaneously in a period of three months. Cases have been described in the literature with producing and non-producing masses of metanephrines. Within these reports, associations with cavernous haemangioma, medulloblastoma, colon cancer, paraganglioma, pheochromocytoma, uterine fibroids, among others were found. However, no association with adrenal masses which do not produce metanephrines was found. In this context, we reported the case of a woman with this type of tumour associated with RCVS which provided a treatment challenge, as well as we reviewed the literature on cases of RCVS associated with masses.

Reversible Cerebral Vasoconstriction Syndrome in the Setting of COVID-19 and Pleomorphic Sarcoma

“…12 In addition, it has been reported in the setting of nonmetanephrine producing tumors or their treatment such as: breast cancer, colon cancer, uterine fibroids, and medulloblastoma. 7 By the time of reporting this case, there have been no cases of RCVS associated with sarcoma and our case is the first one to describe such association. Although there is no clear pathophysiological relationship between these nonfunctional tumors and RCVS, oncological diseases, the noncatecholamine producing ones should be considered as a potential risk factor to decrease the rate of underdiagnosis and to lead to earlier treatment and better prognosis.…”

“…RCVS has been described in the past in the setting of metanephrine-producing masses, especially pheochromocytoma and paraganglioglioma6 proposing that earlier diagnosis of these catecholamines producing tumors has the potential for reducing neurological morbidity and mortality as RCVS is a serious but reversible and treatable cerebrovascular manifestations of these tumors 12. In addition, it has been reported in the setting of nonmetanephrine producing tumors or their treatment such as: breast cancer, colon cancer, uterine fibroids, and medulloblastoma 7. By the time of reporting this case, there have been no cases of RCVS associated with sarcoma and our case is the first one to describe such association.…”

“…Dakay et al 5 described the first and only case report of RCVS complicated by convexal subarachnoid hemorrhage in the setting of COVID-19 infection in a female patient who presented with severe cough and thunderclap headaches, and suggested a possible association between RCVS and SARS-CoV-2 infection. In addition, multiple published cases related the presence of tumor or their treatment with RCVS and suggested that tumors should be considered as a potential risk factor for developing RCVS although some of these tumors are nonfunctional, 7 but none has been published so far in the setting of sarcoma.…”

Reversible cerebral vasoconstriction syndrome (RCVS): an interesting case report

“…To the best of our knowledge, three cases of TCH associated with pheochromocytoma [35][36][37], and four cases of RCVS accompanied by pheochromocytoma [35,[38][39][40] have been reported in the literature. This emphasizes the need of further investigation of the relationship of RCVS and TCH with pheochromocytoma and excessive catecholamine production indicating the importance of including pheochromocytoma in the differential diagnosis of the underlying causes of RCVS or thunderclap headache, especially in case of recurrence, even if the initial investigations are unremarkable.…”

Reversible cerebral vasoconstriction syndrome: literature review

“…The largest studies suggest that 50-80% of patients will have a secondary cause for RCVS [1,10,19,35,41,44]. The main precipitating factors described are: postpartum state [7,10,13,14,16,25,31,35,39,[45][46][47], drugs with vasoactive properties [10, 12-14, 36, 39, 47, 48], such as cocaine, marijuana, and heroin [33,39], catecholaminesecreting tumors [35,[49][50][51], autoimmune disorders, such as systemic vasculitis, systemic lupus erythematous, antiphospholipid syndrome [13], blood transfusions, ginseng [5,6], sexual intercourse, temperature differences (baths too hot or too cold), air travel [39,48], Corona Virus Disease 2019 (COVID-19) infection [36,[52][53][54] and medications [6,29,39,48].…”