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CC BY 4.0 · World J Nucl Med 2023; 22(04): 310-315
DOI: 10.1055/s-0043-1777695
Case Report

Middle Ear Cavity and Mastoid Neuroendocrine Tumor Presenting as Otomastoiditis with Cholesteatoma: A Clinicoradiological and Histopathological Correlation

Ashwini Chalikandy 
1   Radiation Medicine Centre, Bhabha Atomic Research Centre, Tata Memorial Hospital Annexe, Parel, Mumbai, Maharashtra, India
2   Homi Bhabha National Institute, Mumbai, Maharashtra, India
,
Sandip Basu 
1   Radiation Medicine Centre, Bhabha Atomic Research Centre, Tata Memorial Hospital Annexe, Parel, Mumbai, Maharashtra, India
2   Homi Bhabha National Institute, Mumbai, Maharashtra, India
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Abstract

Neuroendocrine tumors of the middle ear are rare, comprising of less than 2% of primary tumors of the ear. The clinical and imaging findings of these tumors are nonspecific, and histological and immunohistochemical findings are confirmatory. Herein, we present a case of 48-year-old male, presenting with chief complaints of hearing loss of left ear with foul smelling discharge, with the initial clinical impression of otomastoiditis of the middle ear with cholesteatoma and being operated for the same, the final histopathology report inferred it as well-differentiated neuroendocrine tumor grade 1 with Ki-67 index less than 2%. Immunohistochemical examinations demonstrated positive staining of the tumor cells for cytokeratin, synaptophysin and chromogranin A, and negative for smooth muscle actin, desmin, S-100. The biochemical investigations showed raised serum chromogranin A levels. Based upon the findings on anatomical imaging modalities including high-resolution computed tomography temporal bone and magnetic resonance imaging paranasal sinuses (MRI PNS), the lesion was inferred inoperable due to involvement of dura of petrous apex, and therefore he was referred for consideration of peptide receptor radionuclide therapy (PRRT). MRI PNS also showed involvement of the horizontal part of facial nerve, indicating local aggressiveness of the tumor. 68Ga-DOTATATE-PET/CT showed high-grade somatostatin receptor expressing soft tissue lesion involving middle ear and external auditory canal (Krenning's score 4), with low-grade metabolic activity on 18F-FDG-PET/CT. The post-therapy scan following 177Lu-DOTATATE PRRT, showed abnormal tracer concentration at the described site. Due to extreme rarity of this disease entity, it is important to accrue data for accurate diagnosis, proper management, and follow-up.

Keywords

neuroendocrine tumor - middle ear cavity - 68Ga-DOTATATE-PET/CT - 177Lu-DOTATATE PRRT


Publication History

Article published online:
26 December 2023

© 2023. The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution License, permitting unrestricted use, distribution, and reproduction so long as the original work is properly cited. (https://creativecommons.org/licenses/by/4.0/)

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  • References

  • 1 Reichart P, Sciubba JJ, Philipsen HP. Splitters or lumpers: the 2017 WHO classification of head and neck tumours. J Am Dent Assoc 2018; 149 (07) 567-571
    CrossrefPubMedGoogle Scholar
  • 2 Sah S, Borkar PV, Wight C, Kelly P, Park KJ, McCluggage WG. Low-grade neuroendocrine tumor of the cervix: report of 3 cases of a rare neoplasm with review of the literature. Int J Gynecol Pathol 2022; 41 (05) 437-446
    CrossrefPubMedGoogle Scholar
  • 3 Makihara N, Maeda T, Nishimura M. et al. Large cell neuroendocrine carcinoma originating from the uterine endometrium: a report on magnetic resonance features of 2 cases with very rare and aggressive tumor. Rare Tumors 2012; 4 (03) e37
    CrossrefPubMedGoogle Scholar
  • 4 Lubana SS, Singh N, Chan HC, Heimann D. Primary neuroendocrine tumor (carcinoid tumor) of the testis: a case report with review of literature. Am J Case Rep 2015; 16: 328-332
    CrossrefPubMedGoogle Scholar
  • 5 Rosen LE, Gattuso P. Neuroendocrine tumors of the breast. Arch Pathol Lab Med 2017; 141 (11) 1577-1581
    CrossrefPubMedGoogle Scholar
  • 6 Wang XH, Lu X, He B. et al. [Clinicopathologic features of primary renal neuroendocrine carcinoma]. Zhonghua Bing Li Xue Za Zhi 2018; 47 (11) 851-856 Chinese.
    PubMedGoogle Scholar
  • 7 Zhou HH, Liu LY, Yu GH. et al. Analysis of clinicopathological features and prognostic factors in 39 cases of bladder neuroendocrine carcinoma. Anticancer Res 2017; 37 (08) 4529-4537
    Google Scholar
  • 8 Said-Al-Naief N, Sciandra K, Gnepp DR. Moderately differentiated neuroendocrine carcinoma (atypical carcinoid) of the parotid gland: report of three cases with contemporary review of salivary neuroendocrine carcinomas. Head Neck Pathol 2013; 7 (03) 295-303
    CrossrefPubMedGoogle Scholar
  • 9 Katabi N. Neuroendocrine neoplasms of the ear. Head Neck Pathol 2018; 12 (03) 362-366
    CrossrefPubMedGoogle Scholar
  • 10 Bell D, El-Naggar AK, Gidley PW. Middle ear adenomatous neuroendocrine tumors: a 25-year experience at MD Anderson Cancer Center. Virchows Arch 2017; 471 (05) 667-672
    CrossrefPubMedGoogle Scholar
  • 11 Guidi M, Buccoliero A, Trabalzini F. Middle ear neuroendocrine tumor: a case report and review of the literature in pediatric population. J Int Adv Otol 2021; 17 (02) 150-155
    CrossrefPubMedGoogle Scholar
  • 12 Yadav SK, Naeem R, Sharma A, Singh S, Sarin N, Pruthi SK. Middle ear adenoma with neuroendocrine differentiation: Report of a rare case. Indian J Cancer 2020; 57 (01) 98-101
    CrossrefPubMedGoogle Scholar
  • 13 Lima Ferreira J, Marques B, Menke-van der Houven van Oordt CW, de Herder WW, Brabander T, Hofland J. Treatment with somatostatin analogues and PRRT in metastatic middle ear adenoma with neuroendocrine features. Endocrinol Diabetes Metab Case Rep 2021; 2: 20-172
    Google Scholar
  • 14 Bruschini L, Canelli R, Cambi C, Fiacchini G, Berrettini S, Forli F. Middle ear neuroendocrine adenoma: a case report and literature review. Case Rep Otolaryngol 2020; 8863188
    PubMedGoogle Scholar
  • 15 Asa SL, Arkun K, Tischler AS. et al. Middle ear “Adenoma”: a neuroendocrine tumor with predominant L cell differentiation. Endocr Pathol 2021; 32 (04) 433-441
    CrossrefPubMedGoogle Scholar
  • 16 Saliba I, Evrard AS. Middle ear glandular neoplasms: adenoma, carcinoma or adenoma with neuroendocrine differentiation: a case series. Cases J 2009; 2: 6508
    CrossrefPubMedGoogle Scholar
  • 17 Marinelli JP, Cass SP, Mann SE. et al. Adenomatous neuroendocrine tumors of the middle ear: a multi-institutional investigation of 32 cases and development of a staging system. Otol Neurotol 2018; 39 (08) e712-e721
    CrossrefPubMedGoogle Scholar
  • 18 Skoura E, Michopoulou S, Mohmaduvesh M. et al. The impact of 68Ga-DOTATATE PET/CT imaging on management of patients with neuroendocrine tumors: experience from a national referral center in the United Kingdom. J Nucl Med 2016; 57 (01) 34-40
    CrossrefPubMedGoogle Scholar
  • 19 van der Lans RJL, Engel MSD, Rijken JA. et al. Neuroendocrine neoplasms of the middle ear: unpredictable tumor behavior and tendency for recurrence. Head Neck 2021; 43 (06) 1848-1853
    CrossrefPubMedGoogle Scholar
  • 20 Yang L, Asiry S, Khader SN. Adenomatous neuroendocrine tumors of the middle ear (MEANTs) with local invasion and lymph nodes metastasis: a brief report of a rare neoplasm. Diagn Cytopathol 2021; 49 (04) E141-E145
    CrossrefPubMedGoogle Scholar
  • 21 Mooney EE, Dodd LG, Oury TD, Burchette JL, Layfield LJ, Scher RL. Middle ear carcinoid: an indolent tumor with metastatic potential. Head Neck 1999; 21 (01) 72-77
    CrossrefPubMedGoogle Scholar
  • 22 Menezes G, Wakely Jr P. Aspiration cytopathology of middle-ear neuroendocrine carcinoma. Diagn Cytopathol 2001; 25 (03) 168-171
    CrossrefPubMedGoogle Scholar
  • 23 Pellini R, Ruggieri M, Pichi B, Covello R, Danesi G, Spriano G. A case of cervical metastases from temporal bone carcinoid. Head Neck 2005; 27 (07) 644-647
    CrossrefPubMedGoogle Scholar
  • 24 Fundakowski CE, Chapman JR, Thomas G. Middle ear carcinoid with distant osseous metastasis. Laryngoscope 2013; 123 (03) 779-782
    CrossrefPubMedGoogle Scholar
  • 25 Gaafar A, Ereño C, Ignacio López J. et al. Middle-ear carcinoid tumor with distant metastasis and fatal outcome. Hematol Oncol Stem Cell Ther 2008; 1 (01) 53-56
    CrossrefPubMedGoogle Scholar