Annals of Clinical Cytology and Pathology

Extracranial Clear Cell Meningioma in Neck: Report of a Rare Case with Diagnostic Challenge to the Cytopathologist

Case Report | Open Access

  • 1. Department of Pathology, Maulana Azad Medical College, India
  • 2. Department of ENT, CNBC, India
+ Show More - Show Less
Corresponding Authors
Nidhi Mahajan, Department of Pathology, Maulana Azad Medical College, A7A/14 Rana Pratap Bagh, Delhi 110007, India, Tel no: 91-9899902074

Extracranial clear cell meningioma is a rare, distinctive and intrinsically aggressive subset of meningioma affecting younger females. The early recognition of this variant is important as it is associated with a high recurrence rate & mortality. We present a case of a 27 year old female who presented with a swelling in the right infra-auricular region. Fine Needle Aspiration Cytology (FNAC) showed pattern less sheets and focal acinar clusters of polygonal cells with vacuolated cytoplasm. A wide differential diagnosis was considered (metastatic renal cell carcinoma, nodular hidradenoma, acinic cell carcinoma, oncocytoma, parathyroid adenoma & sweat gland carcinoma). A clinico-radiological & histopathological correlation was advised. Biopsy showed sheets & whorls of clear round to ovoid cells which were positive for CK, EMA, Vimentin and NSE. Computed tomography scans were reviewed and showed an intracranial extension of the lesion. Based on the above features, a diagnosis of clear cell meningioma was made. This case highlights the need for incorporating clear cell meningioma as a differential diagnosis whenever a clear cell epithelial neoplasm is encountered on FNAC, considering each differential has a distinctive behaviour, treatment protocol and prognosis.


Mahajan N, Sharma D, Khurana N, Jain S, Dhingra S (2015) Extracranial Clear Cell Meningioma in Neck: Report of a Rare Case with Diagnostic Challenge to the Cytopathologist. Ann Clin Cytol Pathol 1(1): 1005.


•    Meningioma
•    Clear cell variant
•    Neck mass
•    Extracranial extension


CCM: Clear Cell Meningioma; FNAC: Fine Needle Aspiration Cytology; IHC: Immunohistochemistry; ICC: Immunocytochemistry; CK: Cytokeratin; EMA: Epithelial Membrane Antigen; NSE: Neuron Specific Enolase


Meningiomas are frequently encountered intracranial tumors occurring throughout the craniospinal axis. These tumors are dura-based and usually found along the external surface of brain and rarely within the ventricular system. Ectopic or extracranial presentation of meningiomas is very rare and located entirely outside the craniospinal confines, usually encountered in the head & neck region, skin, lung, retroperitoneum, mediastinum, foot and external auditory canal [1,2]. The clinicians and the cytopathologists may encounter a diagnostic difficulty when these ectopic meningiomas present at uncommon sites with an uncommon histology. We report a case of Clear Cell Meningioma (CCM) in the posterior triangle of neck, missed on Fine Needle Aspiration Cytology (FNAC) but subsequently diagnosed on histopathology & Immunohistochemistry (IHC). A wide differential diagnosis on FNA smears and the use of Immunocytochemistry (ICC) can help us arrive at a specific diagnosis when one encounters morphological mimics.


A 22 year old female presented to the ENT clinic with a gradually progressive swelling in the posterior aspect of neck for the past 7 months. The swelling was associated with pain, muffling of voice and difficulty in swallowing. No history of blurring of vision or neurological deficit was present. On examination, a 6 X 7cm swelling was seen in the right posterior triangle of neck, with a bulge in the oral cavity and pushing the tonsil to the left side (Figure1A, 1B). FNAC was performed using a 22 gauge needle and yielded a blood mixed aspirate. The smears were stained with May Grunwald Giemsa and showed moderate to high cellularity comprising of round to polygonal cells with moderate amount of vacuolated cytoplasm & minimal nuclear pleomorphism. The cells were arranged in diffuse sheets and focal attempted acinar pattern (Figure 2A, 2B). No necrosis or mitotic figure was seen. A wide differential diagnosis was considered (metastatic renal cell carcinoma, nodular hidradenoma, acinic cell carcinoma, oncocytoma, parathyroid adenoma & sweat gland carcinoma). A clinico-radiological and an excision of the lesion for histopathological correlation were advised. CECT scan revealed a 7 X 6 cm homogenously enhancing soft tissue mass in the right posterior cervical space, displacing the parapharyngeal space anteriorly and superiorly causing destruction of the right temporal bone with intracranial extension (Figure 2C, 2D). Possibility of a malignant neoplastic mass was suggested. A biopsy was performed and it was received in multiple bits together measuring 1 X 0.6 X 0.5 cms. Hematoxylin & eosin stained sections showed round to polygonal cells in a prominent whorling pattern. The nuclei of these cells were round to oval, vesicular with mild to moderate pleomorphism. No mitosis was seen (Figure 3A). In many areas, these cells showed cytoplasmic clearing (Figure 3B). A provisional diagnosis of meningioma was considered and immunohistochemistry for Cytokeratin (CK), Vimentin, Epithelial Membrane Antigen (EMA), Synaptophysin and Neuron Specific Enolase (NSE) was performed. The cells were strongly positive for EMA (Figure 3C), NSE, Vimentin (Figure 3D), focally expressed CK and negative for Synaptophysin. In view of the radiological, histopathological and IHC findings, a definitive diagnosis of clear cell meningioma was given. Surgical excision was planned with the neurosurgical team; however the patient refused to permit the procedure.


Meningiomas account for 24% of primary neoplasms of the central nervous system [3]. Although, most meningiomas are limited to the intracranial space and attached to the duramater, few may extend extracranially, lying completely outside the craniospinal confines (ectopic meningioma). Some intracranial meningiomas may behave poorly and permeate the neighbouring skull to present as skull masses, however maintaining their intracranial connections (secondary meningiomas) [4]. Various mechanisms have been described for the formation of extracranial meningiomas: direct extension of an intracranial mass, origin from arachnoid cells within the sheaths of cranial nerves, origin from embryonic rests of arachnoid cells and metastasising meningioma [5]. Meningiomas usually present in middle or late adult life and show moderate female preponderance (female to male ratio – 3:2)[4].

Although most meningiomas are benign, they have a surprisingly broad spectrum of clinical characteristics and histologically distinct subsets with variable risk of recurrence & prognosis. Classical meningiomas display a wide variety of cytohistological patterns, of which whorl formation is a characteristic feature and evident at least focally. WHO has defined 16 various histologic types of meningiomas based on their risk of recurrence and aggressive behaviour [6]. There are three types of meningiomas according to their grade of malignancy: benign (WHO grade I), atypical (WHO grade II) and anaplastic (WHO grade III)[7].

Clear cell meningioma is a rare subset of meningothelial neoplasms and despite its bland histological appearance shows a potential aggressive behaviour. Zorludemir et al first described this neoplasm in 1995 and it was subsequently listed as a distinct entity in the WHO classification of brain tumors in 2000 [7]. It is associated with a greater likelihood of recurrence and is classified as one of the WHO Grade II tumors. Most CCMs present in the first three decades of life, usually extra axial and often found in the spinal canal, cerebellopontine angle and foramen magnum. In our case, the site of the lesion and the unusual morphology misled the diagnosis. The differential diagnosis of clear cell lesions in the neck includes metastatic renal cell carcinoma, nodular hidradenoma, acinic cell carcinoma, oncocytoma, parathyroid adenoma and myoepithelial variant of sweat gland carcinoma.

Metastatic renal cell carcinoma presents with hematuria, flank pain and a renal mass. Cytology shows large round to polyhedral cells arranged in tubular & acinar pattern, with large vesicular nucleus and prominent nucleoli [4]. Nodular hidradenoma presents as subcutaneous cystic swellings containing clear cells admixed with squamoid cells [4]. Sweat gland carcinomas are usually adenocarcinomas, few of which show signet ring cells. Parathyroid adenomas show a varied morphology comprising of cohesive clusters, disorganised sheets, microfollicles and pseudopapillary fragments. The cells are small, round to oval with pale blue finely vacuolated cytoplasm and a coarse nuclear chromatin [8]. Acinic cell carcinoma shows round to polygonal cells in a glandular or lobular pattern with abundant fine granular cytoplasm. Periodic acid schiff with diastase (PAS- D) highlights the secretory granular cytoplasm of these cells [9]. Oncocytoma is characterised by cohesive clusters and sheets of oncocytes-large cells with abundant granular, eosinophilic cytoplasm with central too eccentric nucleus and prominent nucleolus [10].

The treatment for clear cell meningioma is surgical resection with radiotherapy being reserved for recurrent cases.

To conclude, this report documents an uncommon variant of meningioma presenting as a neck mass and posing a diagnostic difficulty for both the clinician and cytopathologist. It also emphasises the need for incorporating clear cell meningioma as a differential diagnosis whenever a clear cell epithelial neoplasm is encountered on FNAC. Although this tumor is unusual, the characteristic cytological features and appropriate immunocytochemistry if applied can help establish an early definitive diagnosis.


Nidhi Mahajan: Preparation of the manuscript

Divya Sharma: Collection of data and images

Nita Khurana& Shyama Jain: Editing manuscript

Shruti Dhingra: Clinical work up & follow up


1. Marthandapillai A, Alappat JP. Ectopic meningioma: a case report. Neurol India. 2000; 48: 94-95.

2. Kumar G, Basu S, Sen P, Kamal SA, Jiskoot PM. Ectopic meningioma: a case report with a literature review. Eur Arch Otorhinolaryngol. 2006; 263: 426-429.

3. Surawicz TS, McCarthy BJ, Kupelian V, Jukich PJ, Bruner JM, Davis FG. Descriptive epidemiology of primary brain and CNS tumors: results from the Central Brain Tumor Registry of the United States, 1990- 1994. Neuro Oncol. 1999; 1: 14-25.

4. Jayasree K, Divya K. The cytology of intracranial clear cell meningioma with an unusual scalp presentation. J Cytol. 2011; 28: 117-120.

5. Deshmukh SD, Rokade VV, Pathak GS, Nemade SV, Ashturkar AV. Primary extra-cranial meningioma in the right submandibular region of an 18-year-old woman: a case report. J Med Case Rep. 2011; 5: 271.

6. Riemenschneider MJ, Perry A, Reifenberger G. Histological classification and molecular genetics of meningiomas. Lancet Neurol. 2006; 5: 1045-1054.

7. Deb P, Datta SG. An unusual case of clear cell meningioma. J Cancer Res Ther. 2009; 5: 324-327.

8. Absher KJ, Truong LD, Khurana KK, Ramzy I. Parathyroid cytology: avoiding diagnostic pitfalls. Head Neck. 2002; 24: 157-164.

9. Munteanu MC, M?rg?ritescu C, Cionca L, Ni?ulescu NC, D?guci L, Ciuc? EM. Acinic cell carcinoma of the salivary glands: a retrospective clinicopathologic study of 12 cases. Rom J Morphol Embryol. 2012; 53: 313-320.

10. Chakrabarti I, Basu A, Ghosh N. Oncocytic lesion of parotid gland: A dilemma for cytopathologists. J Cytol. 2012; 29: 80-82.

Received : 10 Mar 2015
Accepted : 29 Mar 2015
Published : 30 Mar 2015
Annals of Otolaryngology and Rhinology
ISSN : 2379-948X
Launched : 2014
JSM Schizophrenia
Launched : 2016
Journal of Nausea
Launched : 2020
JSM Internal Medicine
Launched : 2016
JSM Hepatitis
Launched : 2016
JSM Oro Facial Surgeries
ISSN : 2578-3211
Launched : 2016
Journal of Human Nutrition and Food Science
ISSN : 2333-6706
Launched : 2013
JSM Regenerative Medicine and Bioengineering
ISSN : 2379-0490
Launched : 2013
JSM Spine
ISSN : 2578-3181
Launched : 2016
Archives of Palliative Care
ISSN : 2573-1165
Launched : 2016
JSM Nutritional Disorders
ISSN : 2578-3203
Launched : 2017
Annals of Neurodegenerative Disorders
ISSN : 2476-2032
Launched : 2016
Journal of Fever
ISSN : 2641-7782
Launched : 2017
JSM Bone Marrow Research
ISSN : 2578-3351
Launched : 2016
JSM Mathematics and Statistics
ISSN : 2578-3173
Launched : 2014
Journal of Autoimmunity and Research
ISSN : 2573-1173
Launched : 2014
JSM Arthritis
ISSN : 2475-9155
Launched : 2016
JSM Head and Neck Cancer-Cases and Reviews
ISSN : 2573-1610
Launched : 2016
JSM General Surgery Cases and Images
ISSN : 2573-1564
Launched : 2016
JSM Anatomy and Physiology
ISSN : 2573-1262
Launched : 2016
JSM Dental Surgery
ISSN : 2573-1548
Launched : 2016
Annals of Emergency Surgery
ISSN : 2573-1017
Launched : 2016
Annals of Mens Health and Wellness
ISSN : 2641-7707
Launched : 2017
Journal of Preventive Medicine and Health Care
ISSN : 2576-0084
Launched : 2018
Journal of Chronic Diseases and Management
ISSN : 2573-1300
Launched : 2016
Annals of Vaccines and Immunization
ISSN : 2378-9379
Launched : 2014
JSM Heart Surgery Cases and Images
ISSN : 2578-3157
Launched : 2016
Annals of Reproductive Medicine and Treatment
ISSN : 2573-1092
Launched : 2016
JSM Brain Science
ISSN : 2573-1289
Launched : 2016
JSM Biomarkers
ISSN : 2578-3815
Launched : 2014
JSM Biology
ISSN : 2475-9392
Launched : 2016
Archives of Stem Cell and Research
ISSN : 2578-3580
Launched : 2014
Annals of Clinical and Medical Microbiology
ISSN : 2578-3629
Launched : 2014
JSM Pediatric Surgery
ISSN : 2578-3149
Launched : 2017
Journal of Memory Disorder and Rehabilitation
ISSN : 2578-319X
Launched : 2016
JSM Tropical Medicine and Research
ISSN : 2578-3165
Launched : 2016
JSM Head and Face Medicine
ISSN : 2578-3793
Launched : 2016
JSM Cardiothoracic Surgery
ISSN : 2573-1297
Launched : 2016
JSM Bone and Joint Diseases
ISSN : 2578-3351
Launched : 2017
JSM Bioavailability and Bioequivalence
ISSN : 2641-7812
Launched : 2017
JSM Atherosclerosis
ISSN : 2573-1270
Launched : 2016
Journal of Genitourinary Disorders
ISSN : 2641-7790
Launched : 2017
Journal of Fractures and Sprains
ISSN : 2578-3831
Launched : 2016
Journal of Autism and Epilepsy
ISSN : 2641-7774
Launched : 2016
Annals of Marine Biology and Research
ISSN : 2573-105X
Launched : 2014
JSM Health Education & Primary Health Care
ISSN : 2578-3777
Launched : 2016
JSM Communication Disorders
ISSN : 2578-3807
Launched : 2016
Annals of Musculoskeletal Disorders
ISSN : 2578-3599
Launched : 2016
Annals of Virology and Research
ISSN : 2573-1122
Launched : 2014
JSM Renal Medicine
ISSN : 2573-1637
Launched : 2016
Journal of Muscle Health
ISSN : 2578-3823
Launched : 2016
JSM Genetics and Genomics
ISSN : 2334-1823
Launched : 2013
JSM Anxiety and Depression
ISSN : 2475-9139
Launched : 2016
Clinical Journal of Heart Diseases
ISSN : 2641-7766
Launched : 2016
Annals of Medicinal Chemistry and Research
ISSN : 2378-9336
Launched : 2014
JSM Pain and Management
ISSN : 2578-3378
Launched : 2016
JSM Women's Health
ISSN : 2578-3696
Launched : 2016
Clinical Research in HIV or AIDS
ISSN : 2374-0094
Launched : 2013
Journal of Endocrinology, Diabetes and Obesity
ISSN : 2333-6692
Launched : 2013
Journal of Substance Abuse and Alcoholism
ISSN : 2373-9363
Launched : 2013
JSM Neurosurgery and Spine
ISSN : 2373-9479
Launched : 2013
Journal of Liver and Clinical Research
ISSN : 2379-0830
Launched : 2014
Journal of Drug Design and Research
ISSN : 2379-089X
Launched : 2014
JSM Clinical Oncology and Research
ISSN : 2373-938X
Launched : 2013
JSM Bioinformatics, Genomics and Proteomics
ISSN : 2576-1102
Launched : 2014
JSM Chemistry
ISSN : 2334-1831
Launched : 2013
Journal of Trauma and Care
ISSN : 2573-1246
Launched : 2014
JSM Surgical Oncology and Research
ISSN : 2578-3688
Launched : 2016
Annals of Food Processing and Preservation
ISSN : 2573-1033
Launched : 2016
Journal of Radiology and Radiation Therapy
ISSN : 2333-7095
Launched : 2013
JSM Physical Medicine and Rehabilitation
ISSN : 2578-3572
Launched : 2016
Annals of Clinical Pathology
ISSN : 2373-9282
Launched : 2013
Annals of Cardiovascular Diseases
ISSN : 2641-7731
Launched : 2016
Journal of Behavior
ISSN : 2576-0076
Launched : 2016
Annals of Clinical and Experimental Metabolism
ISSN : 2572-2492
Launched : 2016
Clinical Research in Infectious Diseases
ISSN : 2379-0636
Launched : 2013
JSM Microbiology
ISSN : 2333-6455
Launched : 2013
Journal of Urology and Research
ISSN : 2379-951X
Launched : 2014
Journal of Family Medicine and Community Health
ISSN : 2379-0547
Launched : 2013
Annals of Pregnancy and Care
ISSN : 2578-336X
Launched : 2017
JSM Cell and Developmental Biology
ISSN : 2379-061X
Launched : 2013
Annals of Aquaculture and Research
ISSN : 2379-0881
Launched : 2014
Clinical Research in Pulmonology
ISSN : 2333-6625
Launched : 2013
Journal of Immunology and Clinical Research
ISSN : 2333-6714
Launched : 2013
Annals of Forensic Research and Analysis
ISSN : 2378-9476
Launched : 2014
JSM Biochemistry and Molecular Biology
ISSN : 2333-7109
Launched : 2013
Annals of Breast Cancer Research
ISSN : 2641-7685
Launched : 2016
Annals of Gerontology and Geriatric Research
ISSN : 2378-9409
Launched : 2014
Journal of Sleep Medicine and Disorders
ISSN : 2379-0822
Launched : 2014
JSM Burns and Trauma
ISSN : 2475-9406
Launched : 2016
Chemical Engineering and Process Techniques
ISSN : 2333-6633
Launched : 2013
JSM Allergy and Asthma
ISSN : 2573-1254
Launched : 2016
Journal of Neurological Disorders and Stroke
ISSN : 2334-2307
Launched : 2013
Annals of Sports Medicine and Research
ISSN : 2379-0571
Launched : 2014
JSM Sexual Medicine
ISSN : 2578-3718
Launched : 2016
Annals of Vascular Medicine and Research
ISSN : 2378-9344
Launched : 2014
JSM Biotechnology and Biomedical Engineering
ISSN : 2333-7117
Launched : 2013
Journal of Hematology and Transfusion
ISSN : 2333-6684
Launched : 2013
JSM Environmental Science and Ecology
ISSN : 2333-7141
Launched : 2013
Journal of Cardiology and Clinical Research
ISSN : 2333-6676
Launched : 2013
JSM Nanotechnology and Nanomedicine
ISSN : 2334-1815
Launched : 2013
Journal of Ear, Nose and Throat Disorders
ISSN : 2475-9473
Launched : 2016
JSM Ophthalmology
ISSN : 2333-6447
Launched : 2013
Journal of Pharmacology and Clinical Toxicology
ISSN : 2333-7079
Launched : 2013
Annals of Psychiatry and Mental Health
ISSN : 2374-0124
Launched : 2013
Medical Journal of Obstetrics and Gynecology
ISSN : 2333-6439
Launched : 2013
Annals of Pediatrics and Child Health
ISSN : 2373-9312
Launched : 2013
JSM Clinical Pharmaceutics
ISSN : 2379-9498
Launched : 2014
JSM Foot and Ankle
ISSN : 2475-9112
Launched : 2016
JSM Alzheimer's Disease and Related Dementia
ISSN : 2378-9565
Launched : 2014
Journal of Addiction Medicine and Therapy
ISSN : 2333-665X
Launched : 2013
Journal of Veterinary Medicine and Research
ISSN : 2378-931X
Launched : 2013
Annals of Public Health and Research
ISSN : 2378-9328
Launched : 2014
Annals of Orthopedics and Rheumatology
ISSN : 2373-9290
Launched : 2013
Journal of Clinical Nephrology and Research
ISSN : 2379-0652
Launched : 2014
Annals of Community Medicine and Practice
ISSN : 2475-9465
Launched : 2014
Annals of Biometrics and Biostatistics
ISSN : 2374-0116
Launched : 2013
JSM Clinical Case Reports
ISSN : 2373-9819
Launched : 2013
Journal of Cancer Biology and Research
ISSN : 2373-9436
Launched : 2013
Journal of Surgery and Transplantation Science
ISSN : 2379-0911
Launched : 2013
Journal of Dermatology and Clinical Research
ISSN : 2373-9371
Launched : 2013
JSM Gastroenterology and Hepatology
ISSN : 2373-9487
Launched : 2013
Annals of Nursing and Practice
ISSN : 2379-9501
Launched : 2014
JSM Dentistry
ISSN : 2333-7133
Launched : 2013
Author Information X