{"paper_id":"4afe2e5e-d5d4-4548-8200-af7e7b11ce10","body_text":"Review began\n 02/12/2021 \nReview ended\n 04/18/2021 \nPublished\n 04/20/2021\n© Copyright \n2021\nBeck \net al. This is an open access article\ndistributed under the terms of the\nCreative Commons Attribution License\nCC-BY 4.0., which permits unrestricted\nuse, distribution, and reproduction in any\nmedium, provided the original author and\nsource are credited.\nIntramedullary Endometriosis of the Conus\nMedullaris\nTheodoro B. Beck \n \n, \nMauren Beatriz F. Carbonar \n \n, \nRicardo Hanel \n \n, \nRicardo T. Beck \n \n, \nFrancisco Carbonar \n1.\n Surgery, Maternidade Curitiba, Curitiba, BRA \n2.\n Internal Medicine, Maternidade Curitiba, Curitiba, BRA \n3.\nNeurological Surgery, Baptist Neurological Institute lyerly Neurosurgery, Jacksonville, USA \n4.\n Human Reproduction,\nMaternidade Curitiba, Curitiba, BRA \n5.\n Surgery, Universidade Pontifical Catholic University of Paraná, Curitiba, BRA\nCorresponding author: \nTheodoro B. Beck , \nbeckbtheo@gmail.com\nAbstract\nEndometriosis (EM) is a common gynecological disease characterized by endometrial-like tissue outside the\nuterine cavity. We report a case of intramedullary EM, a rare condition with only seven similar cases\nreported until today. MRI showed a mass-like lesion within the spinal canal at the L1-L2 levels and the\nhistological and immunohistochemical features were characteristic of intraspinal endometriosis (IEM). A\nreview of the relevant literature and a comparison between our case and seven other similar cases were\nmade. Intraspinal EM must be recognized as a potential cause of periodic neurological signs and symptoms\nin young and middle-aged women. Timely intervention and appropriate management can result in control\nof the disease and an improvement in neurological functions.\nCategories:\n Obstetrics/Gynecology, Neurosurgery, Oncology\nKeywords:\n endometriosis, conus medullaris, paresthesia\nIntroduction\nEndometriosis (EM) is one of the most common gynecological diseases needing treatment \n[1]\n. The disease is\ncharacterized by the presence of glands and stroma (endometrial-like tissue) outside the uterine cavity \n[2]\n. It\nis mainly reported within the pelvic cavity, primarily on the pelvic peritoneum, ovaries, and rectovaginal\nseptum, and in rare cases on the diaphragm, pleura, and pericardium \n[3]\n. Intraspinal EM (IEM) is rare and\nhas only been reported a few times in the literature \n[4]\n. In the present article, we report a case of\nintramedullary EM, discuss the clinical aspects, radiological and histopathological features, and review the\nrelevant literature.\nCase Presentation\nA 30-year-old woman presented with sudden onset of lower limb paresthesia which awakened her at night\nand was associated with vertigo. The patient presented with a decrease in lower limb sensation to sharp and\ndull point discrimination bilaterally on physical examination. Strength was mildly weak, no urinary or\nintestinal symptoms were reported, and findings did not correlate with her menstrual cycles. The past\nmorbid history described the patient as nulliparous, menarche at 12 years, regular menstrual cycles, and no\nrelated pain. Gynecological examination and abdomen ultrasound were normal. Magnetic resonance\nimaging (MRI) of the dorsolumbar region showed an intramedullary nodular lesion at the conus medullaris at\nlevels L1 and L2 with a heterogeneous signal. The image was partially isotensive and hypointense in T1 and\nhyperintense in T2, measuring 20 × 13 × 08 mm\n3\n (cephalo-caudal × transverse × ventrodorsal). Symmetrical\nexpansion of the cord was evident, and surrounding medullary edema was also observed. Differential\ndiagnosis included underlying neoplasia. The patient underwent surgical treatment with complete removal\nof the mass from the conus medullaris. The mass was firm, with an avascular appearance; no signs of\nprevious bleeding were identified. After removal of the mass, spinal instrumentation was necessary, with no\nfurther injury to the cord and surrounding nerve roots. Gross examination of the surgical specimen revealed\nnodular tissue composed of smooth muscle bundles and cystic glands. Histological sections demonstrated\nthe presence of endometrial stroma and glandular epithelial tissue surrounded by fibro adipose tissue.\nImmunohistochemistry showed CD10 expression by stromal cells, PAX8 by epithelial tissue, and estrogen\nreceptors in stromal and epithelial cells (Figure \n1\n). Complete improvement of sensory and motor deficits\nwas achieved bilaterally. However, she remained on hormonal therapy for one year with Goserelin (Zoladex).\nA postoperative sagittal MRI scan showed a successful resection of the lesion (Figure \n2\n).\n1\n2\n3\n4\n5\n \n Open Access Case\nReport\n \nDOI:\n 10.7759/cureus.14581\nHow to cite this article\nBeck T B, Carbonar M F, Hanel R, et al. (April 20, 2021) Intramedullary Endometriosis of the Conus Medullaris. Cureus 13(4): e14581. \nDOI\n10.7759/cureus.14581\n\nFIGURE\n 1: Histological section of the tumor sample\nHistological section showing nodular tissue composed of smooth muscle bundles and cystic glands.\nHistological sections demonstrate the presence of endometrial stroma and glandular epithelial tissue\nsurrounded by fibroadipose tissue (A). PAX8 by epithelial tissue and estrogen receptors in stromal and\nepithelial cells (B); immunohistochemistry showed CD10 expression by stromal cells (C).\nFIGURE\n 2: Sagital magnetic resonance imaging\nPostoperative T2 sagittal MRI scan at the levels L1 and L2.\n2021 Beck \net al. Cureus 13(4): e14581. DOI 10.7759/cureus.14581\n2\n of \n4\n\nDiscussion\nEndometriosis is a common gynecological disorder that affects 6% to 15% of women of reproductive age \n[3]\n.\nPresentations are diverse, and the most common symptoms are pelvic pain, dysmenorrhea, and dyspareunia\n[2]\n. EM within the central nervous system is an infrequent condition, with only a handful of cases reported in\nthe spinal cord \n[4]\n. Most cases of IEM presented a mass with hematoma. Thus, the differential diagnosis\nincluded underlying neoplasm or vascular lesion \n[4-7]\n. However, our patient had a spinal mass with no\nevidence of bleeding, and neurologic symptoms had no relation to her menstrual cycles, a presentation that\nmakes this case report unique. The rarity of the disease and lack of specific symptoms increase the difficulty\nof identifying the spinal mass origin \n[5,6]\n. The underlying cause of intramedullary EM of the spinal cord is\nunknown, although several hypotheses exist \n[7]\n. Proposed origins of EM are regurgitation through the\nfallopian tube, extrapelvic dissemination through pelvic veins, lymphatic dissemination, and metaplastic\ndifferentiation of coelomic epithelium. Anatomically, IEM probably results from the reverse transport of\nendometriotic tissue via Betson’s venous plexus \n[4]\n.\nSeven articles describing similar spinal EM cases were previously published. All cases describe female\npatients with an average age of 31 years, the youngest being 25 years old and the eldest 42 years old. Most\npresentations are described as chronic development of neurological symptoms associated with the\nmenstrual cycle. Most symptoms were reported as lower extremity radiculopathies, and two of the cases also\nreported difficulty voiding. Six cases reported EM located at the conus medullaris and only one within the\nvertebral region. In all cases, the intramedullary nature of the lesion was initially detected by MRI imaging.\nThe histopathology that was confirmed after surgical biopsies was obtained, except for one case published in\n1968, where MRI imaging was not available \n[8]\n. Patients with lesions located at the conus medullaris were\nsubmitted to laminectomy. However, only half were furtherly treated with medically induced menopause\npost-operatively. The totality of cases reported improvement in their neurological symptoms, and most had\nremission of radicular symptoms. The uniqueness of our case resides in the fact that symptoms started with\nsudden onset of lower limb paresthesia, and symptoms were not associated with menstrual periods,\ncharacteristics that increased the difficulty in making the diagnosis. Another distinguishing characteristic\nwas the absence of hematoma surrounding the lesion in the MRI. While other cases considered vascular\nlesions as a differential diagnosis, the atypical presentation made neoplasia the primary differential\ndiagnosis.\nManagement of intramedullary spinal EM involves both medical and surgical therapies \n[7]\n. Some articles\nthat report similar spinal EM suggest that attempts at total removal of spinal cord EM may be safer after\nachieving pharmacological control \n[7]\n. Our patient etiological diagnosis was only possible after the removal\nof the tumor and histological analysis. Therefore, surgery was performed without prior pharmacological\ntreatment.\nIEM is a rare manifestation of endometriosis, and management is not well established by the literature. Drug\ntherapy can be the initial treatment choice if IEM is suspected \n[9]\n. Patients who present with occupancy of\nthe nidus in the spinal canal might suffer from spinal cord- or cauda equina-related deficits. Patients who do\nnot respond to drug therapy or with frequent recurrence can be managed surgically \n[9]\n. Total removal of the\nspinal cord IEM might be safer after achieving pharmacological control \n[7]\n. Drug therapy post-surgery is also\nan option and should be initiated as soon as confirmation of the diagnosis is made \n[7]\n.\nConclusions\nThis rare case of IEM demonstrates the importance of maintaining a broad differential diagnosis when\nevaluating spinal cord injuries and the necessity of a comprehensive history for each patient. Young female\npatients with acute or menstruation-related neurological symptoms should raise suspicion for IEM. Most\nIEM reported cases are associated with an actively bleeding mass. However, a mass intraspinal lesion\nwithout evident hematoma must also include EM as a differential diagnosis. Moreover, timely intervention\nand appropriate management in patients with neurological symptoms can control the disease and improve\nneurological function.\nAdditional Information\nDisclosures\nHuman subjects:\n Consent was obtained or waived by all participants in this study. \nConflicts of interest:\n In\ncompliance with the ICMJE uniform disclosure form, all authors declare the following: \nPayment/services\ninfo:\n All authors have declared that no financial support was received from any organization for the\nsubmitted work. \nFinancial relationships:\n All authors have declared that they have no financial\nrelationships at present or within the previous three years with any organizations that might have an\ninterest in the submitted work. \nOther relationships:\n All authors have declared that there are no other\nrelationships or activities that could appear to have influenced the submitted work.\nReferences\n1\n. \nEndometriosis: diagnosis and management\n. (2017). 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