Annals of Case Reports (ISSN: 2574-7754)

Article / case report

"Meningioma with Extracranial Pulmonary Metastasis: A Case Report and Literature Review"

Jiawei Wu1#, Zhangqi Dou1#, Yasaman Iranmanesh2, Buyi Zhang4, Yong Hou5, Junxing Wang1, Biao Jiang2*, Zefeng Wang1*,Chongran Sun1*

1Department of Neurosurgery, Second Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, China

2School of Medicine, Zhejiang University, Hangzhou, China

3Department of Radiology, Second Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, China

4Department of Pathology, Second Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, China

5Department of Neurosurgery, Taizhou Hospital of Zhejiang province, Taizhou, China

#Authors Contributed Equally

*Corresponding author(s): Biao Jiang, Department of Radiology, Second Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, China
Zefeng Wang, Department of Neurosurgery, Second Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, China
Chongran Sun, Department of Neurosurgery, Second Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, China

Received Date: 12 October, 2020; Accepted Date: 16 October, 2020; Published Date: 22 October, 2020

Abstract

Meningiomas are usually benign neoplasms in which extracranial metastases occur very rarely. We report a case of an extracranial metastatic tumor diagnosed as a chordoma-like (WHO II) meningioma. A 37-year-old female presented with repeated numbness of the left limbs for two months. Enhanced cranial MRI (Magnetic Resonance Imaging) and MRV (Magnetic Resonance Venous) imaging showed a significantly enhanced tumor that invaded the superior sagittal sinus. To protect the draining vein, we performed a Simpson grade II mass removal, and there was sagittal sinus bleeding during the operation. The histopathologic finding indicated a chordoma-like meningioma. After tumor resection, there was no tumor recurrence.

However, two and a half years later, the patient started to experience substernal pain, and the chest CT scan revealed right lung nodules. To obtain a definite diagnosis, the patient underwent a CT-guided lung biopsy, and the histopathologic findings supported pulmonary metastasis of meningioma. Then, the patient underwent thoracoscopic tumor resection, and after following up the patient by telephone for approximately one and a half years after chest surgery, there was no tumor recurrence of the cerebrum or lung. In conclusion, high-grade meningiomas are prone to metastases, especially those that invade the cranial sinus. Regular follow-up must be performed. Finally, evaluations of the chest, abdomen, and bone are necessary, especially when related symptoms or signs develop.

Keywords

High-grade; Lung/pulmonary; Meningioma; Metastasis; Sinus

Introduction

Meningiomas are slow-growing benign neoplasms that constitute 14% to 19% of all primary intracranial and intraspinal tumors [1]. Although these tumors are typically benign, high-grade meningiomas may occasionally behave aggressively. However, extracranial metastases are very rare, occurring in only 0.1-0.2% of patients [2,3]. Here, we report a case of extracranial metastases to the lung from a chordoma-like (WHO II) intracranial meningioma after cranial tumor resection. Consent was obtained from patients for the retrospective review of their brain MRI, histopathological data and medical records.

Case Information

Clinical Manifestations

A 37-year-old female patient was admitted to our hospital on September 2, 2015, due to repeated numbness of the left limbs. The paralysis of the left limbs was paroxysmal, with each episode lasting more than ten minutes and a frequency of 2-3 times a week.

Imaging Examination

Enhanced cranial MRI (Magnetic Resonance Imaging) and MRV (Magnetic Resonance Venous) imaging (Figure 1) showed that the right parietal mass, which was approximately 6.9 * 5.0 cm in size, was cystic and solid with T1 isointense and T2 hyperintense signals and that the tumor had significant enhancement. The tumor was connected to the meninges with a broad base, and a meningeal tail sign could be seen adjacent to the parenchymal edema; the right ventricle was slightly compressed. The central structure slightly deviated to the left side. The tumor on the right parietal lobe invaded the superior sagittal sinus, the boundary was unclear, a filling defect could be seen behind the posterior superior sagittal sinus, with multiple surrounding collateral vessel shadows. No abnormal signals were found in the bilateral sinus cavities, transverse sinus, and sigmoid sinus. Diagnosis: Meningioma was considered, but hemangioma was not entirely excluded. The tumor on the right parietal lobe invaded the superior sagittal sinus.

Surgical Treatment

A right parietal midline incision was made. During the operation, a tumor was located beneath the meninges, showing a pale yellow color, a tough texture, a size of approximately 7 * 7.5 cm, and abundant blood supply. The base was located on the convex surface of the meninges, the sickle of the brain, and the sagittal sinus wall. Obvious adhesions to the brain tissue were observed. The tumor was mainly removed under a microscope. A thick vein passed from the deep part of the tumor and extended into the sagittal sinus along with the sickle of the brain. To protect the draining vein, a small amount of tumor was retained. After the resection, there was sagittal sinus bleeding, which was caused by the reconstruction of the sagittal sinus using gelfoam and BioGlue. Simpson grade II tumor resection was achieved. Intraoperative frozen pathology results showed meningioma with atypical cells and dense cells in some areas. After the operation, the patient was in good general health, and the numbness of the left limbs did not recur. Cranial CT and MRI showed no abnormalities one week postoperation.

Histopathologic Diagnosis

Postoperative pathology revealed right parietal meningioma with atypical cells, interstitial mucinous degeneration, and occasional mitosis, which was considered to be chordoma-like (hematoxylin-eosin, ×400). The immunohistochemical results were as follows: Ki67 5%, P53 +, EMA +, GFAP-, NSE foci +, CD99-, CD56 foci +, and CK (AE1/AE3) (Figure 2).

Postoperative Follow-up

Follow-up images of enhanced cranial MRI within four years after surgery (Figure 3).

Pulmonary Metastases

Two and a half years after tumor resection, the patient presented with substernal pain without any apparent inducing factors. The chest pain was described as paroxysmal dull pain and was exacerbated with deep breaths. There were no other simultaneous symptoms, such as cough, phlegm, belching or acid regurgitation. The chest CT scan revealed right lung nodule (Figure 2). However, no significant improvement was observed after anti-inflammatory treatment. To obtain a definite diagnosis, CT-guided lung biopsy was performed in our hospital. A total of two gray-white lesions were removed. The histopathologic findings supported pulmonary metastasis of meningioma. After undergoing puncture biopsy in our hospital, the patient underwent thoracoscopic tumor resection in another hospital. The postoperative histopathological report supported the histopathologic description of the puncture biopsy results in our hospital. After following up the patient for approximately one and a half years by telephone, the general medical condition of the patient was acceptable (KPS score 100 points). The patient had no head discomfort, seizures, numbness in the left limbs, or significant chest discomfort.

Discussion

Meningiomas are primary Central Nervous System (CNS) tumors that originate from arachnoid cap cells around the brain [4]. At present, meningioma has surpassed glioma as the most common primary central nervous system tumor [5]. The vast majority of meningiomas are considered benign and can be treated with surgical resection and adjuvant radiation therapy. Compared to other central nervous system tumors, extracranial metastatic meningiomas are extremely rare, occurring in only 0.1-0.2% of patients [2,3]. The organs most likely to develop metastases are the lung (37%), bones (16.5%), spine (15.2%), liver (9.2%), adrenal glands, neck, and other structures (21.9%) [6-11]. Atypical or anaplastic meningioma is prone to extracranial metastasis [12].

According to the 2016 edition of the World Health Organization (WHO) classification of neurological tumors (CNS), the histopathologic classification of 15 meningiomas is listed as follows [13]. The subtypes that are prone to extracranial metastasis are papillary meningioma (WHO II), atypical meningiomas (WHO II), and anaplastic meningiomas (WHO III) [14-16]. Certain imaging features, such as mushroom-like growth, nonuniform enhancement, peritumoral edema, osteolysis, inherent cyst-like areas, and fuzzy tumor-brain borders, are considered to be important clues for the diagnosis of malignant or aggressive tumors [17]. Furthermore, immunohistochemical analysis of nuclear protein, Ki-67 and P53 marker indexes related to cell proliferation or molecular markers such as CDKN2A deletion and 9p21 deletion are very useful for assessing the possibility of tumor recurrence or metastasis [18,19].

The Ki-67 index is significantly elevated in benign meningiomas (mean 3.8%), atypical meningiomas (mean 7.2%) and anaplastic meningiomas (mean 14.7%) [20]. Intracranial lesions usually recur several times locally before metastasis, but in this case, no intracranial recurrence occurred before extracranial metastases [3]. One of the most common chromosomal aberrations of meningiomas is the partial or complete deletion of chromosomes. Somatic mutations of the NF2 gene at 22q12.2 have been described as early events of tumorigenesis [21]. The second most significant genetic change of meningiomas is the partial or complete deletion of 1p, which is also the most common progression-related chromosomal aberration in meningiomas [22]. In related studies, mutations of 22q and 1p in primary intracranial meningiomas and lung metastases were detected. The chromosomal changes in the latter may reflect the chromosomal and genetic heterogeneity of these tumors [23].

The location of meningioma metastases mainly depends on the route of spread. Four different routes of transmission have been proposed [24]: (i) transjugular vein (supported by evidence of cervical lymph node, thyroid, lung/pleural metastasis); (ii) paravertebral venous plexus (“Batterson’s venous drainage”); (iii) tumor invasion and proximity to the vena cava; and (iv) localized metastasis via the lymphatic and cerebrospinal fluid pathways. Among these routes, hematogenous metastasis is mainly caused by tumor cells that invade the venous system and spread through the vena cava system, which may be the reason why the lung and liver are prone to metastasis [25]. Although meningiomas originate from arachnoid cells and are naturally exposed to cerebrospinal fluid during their growth or surgical intervention, metastasis through the cerebrospinal fluid is less common than hematogenous metastases to the internal and external organs [26].

The factors affecting extracranial metastasis include the following: (i) an increase in the degree of malignancy, (ii) tumor recurrence, and (iii) tumor invasion of the venous sinuses [23]. More than 90% of cases of extracranial metastasis reported in related studies occur after resection or shunt surgery. These two processes help tumor cells enter the extrameningeal blood vessels and lymphatic vessels [27]. In this case, the tumor had an abundant blood supply, with a large drainage vein on the brain surface at the anterior margin of the tumor and a vein at the posterior margin that ran deep through the tumor, which might have provided a functional pathway for tumor metastasis. There was also hemorrhage of the sagittal sinus during tumor resection, which might have caused tumor colonization in the lungs along the venous system.

The incidence of extracranial metastases caused by meningiomas is very low, and there is currently no standard treatment for extracranial metastases; however, general surgical resection is preferred [28]. Postoperative radiotherapy has been recommended to prevent local recurrence, especially when resection or histology suggest a malignant tumor [29]. In most published cases of pulmonary metastases, early complete lung tumor resection is the preferred treatment [30-34]. In this case, after the lung tumor was removed, no chemoradiotherapy was performed, and no recurrence of the lung lesions was found, which may be related to the relatively low tumor invasiveness. The prognosis of intracranial and extracranial meningioma metastases is unknown [35].

Conclusion

When meningiomas metastasize or radiologic findings indicate aggressive behavior, careful histopathological examination with immunohistochemistry analyses should be performed to determine the nature of the tumor. In intracranial high-grade meningioma, extracranial metastasis should be considered, especially when the meningioma invades the cranial sinus; regular follow-up is necessary with evaluations of the chest, abdomen and bone, especially when related symptoms or signs also develop.

Disclosure of Funding

This work was supported by the Key Research and Development Project of China (No. 2018YFA0108603), the National Natural Science Foundation of China (No. 81771246 and No. 81870908) and Zhejiang Provincial department of education project in China (NO. Y201636513).

Conflict of Interest

The authors declared no conflict of interest.

Authorship

Research design: CS and ZW; data collection: Yasaman Iranmanesh, BZ and BJ; data analysis: JW (Jiawei Wu), ZD, JW (Junxing Wang) and YH; manuscript writing and revising: JW (Jiawei Wu), ZD and CS. All authors listed above contributed greatly to drafting the manuscript and performing critical revision, and had access to the data and approved the final manuscript for submission. We declare that the work described in this research paper has not been published previously nor been under consideration for publication elsewhere.


Figure 1: A and B: the right parietal mass, which was approximately 6.9 * 5.0 cm in size, was cystic and solid with T1 isointense and T2 hyperintense signals; C-E: the tumor had significant enhancement; F-H: The tumor on the right parietal lobe invaded the superior sagittal sinus, the boundary was unclear, a filling defect could be seen behind the posterior superior sagittal sinus, with multiple surrounding collateral vessel shadows.


Figure 2: A and B: right parietal meningioma with atypical cells, interstitial mucinous degeneration, and occasional mitosis, which was considered to be chordoma-like (hematoxylin-eosin, ×40). The immunohistochemical results were as follows: Ki-67 5%, P53 +, EMA +, GFAP-, NSE foci +, CD99-, CD56 foci +, and CK (AE1/AE3); C: right middle lobe nodules with a maximum diameter of 20mm shadow.


Figure 3: Enhanced cranial MRI was performed after surgery with no visible abnormal changes.


References

  1. Wara WM, Sheline GE, Newman H, Townsend JJ, Boldrey EB (1975) Radiation therapy of meningiomas 123: 453-458.
  2. Schweitzer T, Vince GH, Herbold C, Roosen K, Tonn J-C (2001) Extraneural Metastases of Primary Brain Tumors Journal of Neuro-Oncology 53: 107-114.
  3. Lee G-H, Choi S-W, Kim S-H, Kwon H-J (2009) Multiple extracranial metastases of atypical meningiomas. Journal of Korean Neurosurgical Society 45:107-111.
  4. Riemenschneider MJ, Perry A, Reifenberger G (2006) Histological classification and molecular genetics of meningiomas. Lancet Neurology 5: 1045-1054.
  5. Ostrom QT, Gittleman H, Liao P, Vecchione-Koval T, Wolinsky Y, et al. (2016) CBTRUS Statistical Report: Primary Brain and Other Central Nervous System Tumors Diagnosed in the United States in 2009-2013. Neuro-oncology 19: v1-v88.
  6. Erman T, Hanta I, Hacıyakupoğlu S, Zorludemir S, Zeren H, et al. (2005) Huge Bilateral Pulmonary and Pleural Metastasis from Intracranial Meningioma: A Case Report and Review of the Literature. Journal of Neuro-Oncology 74: 179-181.
  7. Chohan MO, Ryan CT, Singh R, Lanning RM, Reiner AS, et al. (2018) Predictors of Treatment Response and Survival Outcomes in Meningioma Recurrence with Atypical or Anaplastic Histology. Neurosurgery 82: 824-832.
  8. Vik A, Kvikstad A, Unsgård G, Jørgensen JV, Valentin SHT (2006) [A 54-year-old man with a large subcutaneous skull erosion and focal epileptic seizures]. Tidsskr Nor laegeforen 126: 2386-2387.
  9. Fabi A, Nuzzo C, Vidiri A, Ciccarese M, Felici A, et al. (2006) Bone and lung metastases from intracranial meningioma. Anticancer research 26: 3835-3838.
  10. Shawn Teague and Dewey J Conces (2006) Diagnosis of Lung Cancer: Perspective of a Pulmonary Radiologist. PET clinics 1: 289-300.
  11. Surov A, Gottschling S, Bolz J, Kornhuber M, Alfieri A, et al. (2013) Distant metastases in meningioma: an underestimated problem. Journal of Neuro-Oncology 112: 323-327.
  12. Asioli S, Senetta R, Maldi E, D'Ambrosio E, Satolli MA, et al. (2007) “Benign” metastatic meningioma: clinico-pathological analysis of one case metastasising to the lung and overview on the concepts of either primitive or metastatic meningiomas of the lung. Virchows Archiv 450: 591-594.
  13. Louis DN, Perry A, Reifenberger G, Deimling Av, Figarella-Branger D, et al. (2016) The 2016 World Health Organization Classification of Tumors of the Central Nervous System: a summary. Acta Neuropathologica 131: 803-820.
  14. Pramesh CS, Saklani AP, Pantvaidya GH, Heroor AA, Naresh KN, et al. (2003) Benign metastasizing meningioma. Jpn J Clin Oncol 33: 86-88.
  15. Kros JM, Cella F, Bakker S, Paz Y Geuze D, Egeler RJA (2000) Papillary meningioma with pleural metastasis: case report and literature review. Acta Neurol Scand 102: 200-202.
  16. Drummond KJ, Bittar RG, Fearnside MR (2000) Metastatic atypical meningioma: case report and review of the literature. Journal of Clinical Neuroscience 7: 69-72.
  17. New PF, Hesselink JR, O'Carroll CP, Kleinman GM (1982) Malignant meningiomas: CT and histologic criteria, including a new CT sign. AJNR American journal of neuroradiology. 3: 267-276.
  18. Perry A, Banerjee R, Lohse CM, Kleinschmidt-DeMasters BK, Scheithauer BW (2002) A role for chromosome 9p21 deletions in the malignant progression of meningiomas and the prognosis of anaplastic meningiomas. Brain pathology 12: 183-190.
  19. Terzi A, Saglam EA, Barak A, Soylemezoglu F (2008) The significance of immunohistochemical expression of Ki-67, p53, p21, and p16 in meningiomas tissue arrays. Pathology-Research and Practice 204: 305-314.
  20. Maier H, Maier H, Wanschitz J, Sedivy R, Rössler K, et al. (1997) Proliferation and Dna fragmentation in meningioma subtypes. Neuropathology and Applied Neurobiology 23: 496-506.
  21. Campbell BA, Jhamb A, Maguire JA, Toyota B, Ma R (2009) Meningiomas in 2009: controversies and future challenges. American journal of clinical oncology 32: 73-85.
  22. Lopez-Gines C, Cerda-Nicolas M, Gil-Benso R, Callaghan R, Collado M, et al. (2004) Association of loss of 1p and alterations of chromosome 14 in meningioma progression. Cancer Genetics and Cytogenetics 148: 123-128.
  23. Frydrychowicz C, Holland H, Hantmann H, Gradistanac T, Hoffmann KT, et al. (2015) Two cases of atypical meningioma with pulmonary metastases: a comparative cytogenetic analysis of chromosomes 1p and 22 and a review of the literature. Neuropathology: official journal of the Japanese Society of Neuropathology 35: 175-183.
  24. Kanthan R and Senger JL (2007) Distant Metastases from Meningiomas - A Myth or Reality? Ann Clin Pathol 16: 145-162.
  25. VShimanskiĭ VN, Rotin DL, Shishkina LV, Otarashvili IA, Kobiakov GL (2011) [Meningioma with extracranial metastases]. Zhurnal voprosy neirokhirurgii imeni N. N. Burdenko 75: 62-67.
  26. John J Kepes (1983) Meningiomas: Biology, Pathology and Differential Diagnosis, Masson Publishing USA, New York (1982), 206 pages, illustrated. $52.00. Schoene William C 14.
  27. Patrick Beauchesne (2011) Extra-neural metastases of malignant gliomas: myth or reality? Cancers 3: 461-477.
  28. Attuati L, Zaed I, Morselli C, Pecchioli G, Fornari M, et al. (2019) Multimodal Management of Metastatic Malignant Meningiomas: The Role of Radiosurgery in Long-Term Local Control. World Neurosurgery 128: 562-572.
  29. Enam SA, Abdulrauf S, Mehta B, Malik GM, Mahmood A (1996) Metastasis in meningioma. Acta Neurochirurgica 138, 1172-1178.
  30. Nakayama Y, Horio H, Horiguchi S, Hato T (2014) Pulmonary and pleural metastases from benign meningeal meningioma: a case report. Ann Thorac Cardiovasc Surg 20: 410-413.
  31. Brennan C, O'Connor OJ, O'Regan KN, Keohane C, Dineen J, et al. (2010) Metastatic meningioma: positron emission tomography CT imaging findings. Br J Radiol 83: e259-262.
  32. Etienne-Mastroianni B, Girard N, Ginguene C, Tronc F, Vasiljevic A, et al. (2010) [Pulmonary metastases from malignant meningioma]. Revue des maladies respiratoires 27: 764-769.
  33. Fulkerson DH, Horner TG, Hattab EM (2008) Histologically benign intraventricular meningioma with concurrent pulmonary metastasis: case report and review of the literature. Clinical neurology and neurosurgery 110: 416-419.
  34. Hironori I, Ohta S, Hirose M, Furuhashi K, Suzuki M, et al. (2008) [Pulmonary metastatic meningioma 26-years after craniotomy]. Kyobu geka. The Japanese journal of thoracic surgery 61: 478-481.
  35. Daniela A, Glantz Michael J, Kim Lyndon, Chamberlain Marc C, Bota Daniela A (2011) Pulmonary metastases in patients with recurrent, treatment-resistant meningioma: prognosis and identification by ¹¹¹Indium-octreotide imaging. Cancer 117: 4506-4511.

Citation: Wu J, Dou Z, Iranmanesh Y, Zhang B, Hou Y, et al. (2020) Meningioma with Extracranial Pulmonary Metastasis: A Case Report and Literature Review. Ann Case Report 14: 494. DOI: 10.29011/2574-7754.100494

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