Simona Vallarelli1, Stefania Stucci1*, Marco Tucci1, Nicola Quaranta2, Donatella Russo3, Marco Moschetta4 and Franco Silvestris1
1DIMO, Department of Biomedical Sciences and Clinical Oncology University of Bari ‘Aldo Moro’, Italy
2Department of Ophthalmology and Otolaryngology, University of Bari ‘Aldo Moro’, Italy
3Department of Radiation Oncology, V. Fazzi Hospital, Lecce, Italy
4Department of Medicine, Section of Diagnostic Imaging, University of Bari ‘Aldo Moro’, Italy
Received: 25 January, 2016; Accepted: 20 February, 2016; Published: 22 February, 2016
Stefania Stucci, MD, Medical Oncology Unit, DIMO, University of Bari ‘Aldo Moro’, P.za Giulio Cesare, 11 - 70124 Bari, Italy, Tel: +39.080.547.8674; Fax: +39.080.547.8831; E-mail:
Vallarelli S, Stucci S, Tucci M, Quaranta N, Russo D,et al. (2016) Adenocarcinoma of Ceruminous Glands: Role of the VMAT. Arch Otolaryngol Rhinol 2(1): 009-012. DOI: 10.17352/2455-1759.000013
© 2015 Vallarelli S, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
Adenocarcinoma of the ceruminous glands is a rare malignancy arising from the glands of the external auditory canal. In most patients it is usually diagnosed as locally advanced disease with a major obstacle for radical surgery. Here, we describe two cases of patients suffering of sudden hearing loss and ipsilateral facial hemiparesis due to tumors arising from the ceruminous glands with primary intracranial involvement and subsequent middle ear infiltration. The patient with local-advanced disease underwent surgery followed by adjuvant treatment, whereas the other patient with advanced disease only to palliative therapy. However, both of them received volumetric-modulated arc radiotherapy (VMAT) resulting in complete remission as adjuvant treatment in the first patients while extending the survival as palliation in the second one. Thus, VMAT appears a suggested approach in this tumor which management is still poorly defined.
Adenocarcinoma of the ceruminous glands originates from the apocrine sweat glands located within the external auditory canal [1-3]. Its incidence is undefined and the clinical presentation is mostly characterized by general symptoms. Although the diagnosis of malignant ceruminoma is generally provided by the pathologist, both computed tomography (CT) scan and nuclear magnetic resonance (NMR) are commonly utilized for diagnosis [4,5]. The tumor shows poor prognosis and the majority of studies suggest that the best clinical result is provided by the radical excision followed by radiation , based on the ineffectiveness of chemotherapy in terms of clinical response rate (RR) and overall survival (OS). However, it has been experienced that the intensity-modulated radiation therapy (IMRT) such as VMAT (volumetric-modulated arc therapy) is of great effort for the treatment of this tumor in relation to minor side effects and better safey of peritumoral structures as compared to the conformational irradiation . Two patients with malignant ceruminoma were admitted at the Medical Oncology Unit of the University of Bari ‘Aldo Moro’ and both treated with VMAT for adjuvant and palliative treatment.
The patients has given their informed consent for the case report to be published. A 71-year-old male (patient n.1) suffering of headache, left facial nerve hemiparesis and lagophtalmos associated to ipsilateral sudden-hearing loss was admitted in our department. Blood tests were normal and the patient underwent whole-body CT-scan revealing a mass with irregular margins and hypervascularity of 5x5 cm localized nearby the left pre-auricular area and invading the middle cranial fossa with extension up to the external auditory canal and the tympanic cavity as well as to the petrous bone. In addition, the tumor infiltrated the parotid gland leading to medial dislocation of the temporal horn although the ventricular system was not enlarged (Figure 1, panels A,B) whereas the NMR revealed the metastatic local colonization of cervical, submandibular and retro-nuchal nodes. The patient was biopsied in several parts including the mastoid, middle ear, dural tissue and middle cranial fossa whose microscopic examination identified the presence of poorly differentiated spindle cells showing a high number of nucleoli and vacuoli with a trabecular-like organization (panel C). The diagnosis of poorly differentiated adenocarcinoma of the ceruminous gland with meningeal infiltration was confirmed by immunohistochemistry showing the cytokeratin pool, as well as both the epithelial membrane antigen and S100 protein (panels D and E), whereas other ontogenetic markers as glial fibrillary acidic protein, neuron specific enolase (NSE) and epidermal growth factor receptor (EGFR) were not detected . Based on the intracranial extension of the malignant cells, the patient was not eligible for radical surgery and underwent VMAT treatment extended up to five weeks for a total dose of 6000 cGy (panels F,G). This treatment induced a partial response according to the RECIST criteria , that was maintained up to four months with a stable tumor size of 3 cm (panels H,I) in parallel with clinical benefit. The objective RR was maintained for eight months when the patient suffered of sudden weakness of the right arm associated to burning pain of the thoracic spine. New radiological assessments including NMR and both brain and spinal-cord CT-scan confirmed the clinical progression by pathologic tissue with high contrast-enhancement in the extradural and left insulo-parietal level in parallel with infiltration of the skull in the omolateral temporal-occipital region. The spine examination showed the occurrence of metastatic vertebral lesions with intradural infiltration at both cervical and dorsal levels. Moreover, other pathologic tissue involved the right pedicles of D5 and D7 as well as the lumbar spine leptomeninges. Thus, the patient was considered only for best supportive care with monthly bisphosphonates and palliative rehabilitation.
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