Successful stereotactic radiotherapy of meningiomas in a patient with Cowden syndrome: a case report

Abstract

Cowden's Syndrome (CS) is a rare disease with increased risk for several carcinomas.Experimental studies and limited case reports have described the negative effects of radiotherapy. A 35-yearold woman presented with newly diagnosed CS and multiple meningiomas. She underwent subtotal resection of a right petroclival meningioma to relieve brainstem compression and received adjuvant fractionated stereotactic radiotherapy 50 Gy in 25 fractions with minimal side effects. Twenty months post-operatively the patient presented with neurological deficits from progression of additional meningiomas. Craniotomy was performed and gross total resection was achieved for all sites of disease. Imaging five months after surgery demonstrated progressive left tentorial meningioma. She underwent definitive stereotactic radiosurgery to 15 Gy and tolerated treatment well. At 32 and 7 months post-RT, the patient has reported no side effects or toxicity as a result of RT, demonstrating for the first time in the literature, to the best of our knowledge, the use of intracranial RT without significant toxicity in CS.

Coexisting lipomatous meningioma and glioblastoma in Cowden syndrome: A unique tumor association

“…Regarding the histological grade of malignancy of the meningiomas, seven out of the 10 tumors, including ours, providing such information corresponded to WHO grade I. 3,10,11,17,21 The meningioma analyzed in our patient corresponded to a metaplastic subtype showing a lipomatous appearance, a peculiarity never previously reported in a CS patient. This very rare histological characteristic, thought to result from lipid accumulation rather than true lipomatous metaplasia, however, is associated with the same good prognosis as conventional grade I meningiomas.…”

“…12 More than one tumor lineage was reported in six cases, all but one corresponding to meningiomas and concurrent LDD. 3,10,11,14,18 The concomitant intracranial lesions in the other patient were multiple meningiomas and a pituitary adenoma that later progressed to carcinoma. 13 Our patient is the first one ever reported with coexisting meningioma and glioblastoma.…”

“…2 With regard to central nervous system tumors, the association with dysplastic cerebellar gangliocytoma, also known as Lhermitte-Duclos disease (LDD), is the only widely accepted one, thus falling under the major diagnostic criteria for CS. 2,3 The potential relation of CS with other brain tumors is, however, not properly recognized, even though the frequency of meningiomas seems to be higher in CS patients than would be expected on average. [4][5][6] The rarity of CS, in addition to the unfamiliarity of most clinicians with the possible involvement of the intracranial compartment, apart from LDD, prompted us to write this article.…”

“…A thorough literature review of CS patients' intracranial tumors other than LDD, despite the fact that this condition also exists in some cases concurrently, is also presented (Table 1). 1,3,4,[7][8][9][10][11][12][13][14][15][16][17][18][19][20][21][22][23][24] Our major aim is to raise awareness about the association between CS and other types of primary brain tumors.…”

Multiple Meningiomata in Cowden Syndrome

“…In another report, a patient with CS was diagnosed with undifferentiated pleomorphic sarcoma of the breast, thought to be from adjuvant RT for breast cancer seven years prior [13]. Conversely Fernandez et al document the case of a 35-year-old CS patient who underwent SRS at 15Gy which was not only well-tolerated, but at seven months post-RT devoid of side effects or toxicity [14].…”