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Research Article *Corresponding author Julian Küstermeyer, Department of Otorhinolaryngology, Head and Neck , KRH Tumor of the Temporal Nordstadt Clinic, Academic Hospital, Haltenhoffstr, 41, 30167 Hanover, Germany, Tel: 49-511-970-4377; Fax: 49- 511-970-4642; Email: Mimicking Acute Mastoiditis Submitted: 11 April 2015 Julian Küstermeyer1*, Helmut Ostertag2, Jörg Hattingen3 and Accepted: 02 June 2015 Published: 04 June 2015 Hans-Jürgen Welkoborsky1 Copyright 1Department of Otorhinolaryngology, Head and Neck Surgery, KRH Nordstadt Clinic, © 2015 Küstermeyer et al. Germany 2 Department of Pathology, KRH Nordstadt Clinic, Germany OPEN ACCESS 3Department of Radiology, KRH Nordstadt Clinic, Germany Keywords Abstract • Angiosarcoma • Temporal bone Background: Tumors of the temporal bone in general and particular in children • Skull base are extremely rare. The anatomical conditions of that special anatomic region can • Tumor cause misleading symptoms. Diagnosis of rare tumor entities of the skull base in children • Mastoiditis is a challenging interdisciplinary task. Objective: The purpose of the present article is to describe the diagnostic process of a rare pathologic condition and review the literature with current opinions of diagnostic and therapeutic methods with respect to angiosarcomas. Methods: We report about a 13 years old boy with an angiosarcoma of the temporal bone. A review of the literature with particular emphasis on the diagnostic procedures was performed. Results: The clinical and histological characteristics are reported in here. To date less than 20 cases of angiosarcomas of the temporal bone are published in the literature. Although a large variety of diagnostic methods is available, diagnosing and of such a rare tumor is still challenging. Conclusion: Angiosarcoma of the temporal bone is extremely rare. The symptoms are unspecific, and can mimicking, like in this case, an acute mastoiditis. Diagnosis is challenging and only possible by Immunohistochemical examinations. Diagnosis of such rare pathologic conditions requires an interdisciplinary teamwork and collaboration of different institutions. Current technologies allow an easy exchange of knowledge all around the world for characterizing diseases precisely, and getting the experience of specialists together. This is crucial for a targeted and effective treatment.

INTRODUCTION hearing loss. During initial examination the right retro auricular region was not swollen or hyperemic. However, otomicroscopy Otalgia and hearing loss are common reasons for consulting revealed middle-ear effusion of the right ear. Therefore the an ENT physican. In most cases, these symptoms are caused by patient received a tympanic drain on the right side and was acute ear . Children mostly suffer from otitis media treated with parenteral cefotaxime. With this the patient’s status because of anatomical conditions leading to eustachian tube improved quickly. dysfunction [1,2]. Acute mastoiditis is one of the most frequent complication of acute otitis media in children and adults [3]. Some weeks later the patient was readmitted with persistent Classical symptoms are otalgia, , posterior auricular swelling, complaints, now presenting a swelling of the right retro auricular erythema and mastoid tenderness [4,5]. Differential diagnoses region. Furthermore the mastoid was sensitive to percussion. A include insect bites, severe external otitis, or rare causes like computed tomography of the temporal bone showed complete Langerhans histiocytosis and [6]. In this case report we describe a rare tumor entity, which showed symptoms similar to 1). Thus, mastoidectomy was planned. During the surgical mastoiditis. proceduremastoid opacification the mastoid and bone partial appeared bone suspicious. destruction A (Figuresample of the bone tissue was taken and the operation aborted. A MRI CASE PRESENTATION scan of the head revealed skull base destruction by a large mass A 13-year-old boy was admitted to the ENT department in children’s hospital with right sidedotalgia, ear pressure and infiltrating the mastoid bone and the dura. The brain tissue was not affected (Figure 2). However, the clinical status of the patient Cite this article: Küstermeyer J, Ostertag H, Hattingen J, Welkoborsky HJ (2015) Tumor of the Temporal Bone Mimicking Acute Mastoiditis. Ann Otolaryngol Rhinol 2(5): 1039. Küstermeyer et al. (2015) Email: Central was unimpaired. Preliminary results of revealed a kaposiform hemangio endothelioma with highly mitotic activity but without signs for invasive growth pattern, and the differential diagnosis of a sarcomatoid tumor. The patient was hospitalized again because of a bilateral papilledema. Signs of intracranial pressure could be excluded by neurosurgeons. Examinations were completed with , computed tomography of the neck, of the thorax and of the abdomen, along with a digital subtraction angiography no further suspicious . CT scan revealed no pathologic of the skull base region (Figure 3). Bone scintigraphy showed of the head a hypervascularized tumor without an early phase venousfindings perfusion in the neck, was thorax described. and abdomen. It received In anits angiography main supply from the middle meningeal artery.

Figure 3 Angiography of the head showing the hypervascularized tumor.

Pathologists recommended obtaining extended samples

histopathologic diagnosis consiliary examination and statements of reference tumor tissue, centers because were required. of the rareIn order tumor to take entity. a new For sample final an operation together with neurosurgeons was performed. During this procedure three different samples were taken using a retro auricular approach: Soft tissue overlying the skull, destructed skull base, and tumor masses lying next to the dura mater. Microscopic examination revealed spindle cell patterns and areas with numerous erythrocytes within in the tumor, Figure 1 Axial CT scan with complete mastoid attenuation and partial bone destruction. cells with endothelial growth patterns. Immunohistological furthermore small fields with necrosis and also parts of tumor CD34, ERG), rare positivity for vessel markers, especially factor findings showed high positivity for endothelial markers (CD31,

inVIII, cooperation signs for high with proliferative national and activity international (MiB1) andreference negativity centers for HHV8 and S100.The final results of histopathological examination

for endothelial and bone tumors (i.e. Memorial Sloan-Kettering revealedCancer Center an highly New York, proliferative NY, USA; Kiel malignant Pediatric endothelial Tumor Registry, tumor Germany; Bone Tumor Reference Center Basel, Switzerland)

of the skull base with infiltrating the soft tissue. The tumor was finally diagnosed as a high grade angiosarcoma. in a local center for pediatric . The regimenDue to included the tumor cyclophosphamide, extension the patient vincristine, first adriamycinunderwent and paclitaxel. A response evaluation after nine weeks by MRI

However, an interdisciplinary tumor board recommended a furtherrevealed conservative a significant treatment reduction with of additional tumor mass radiation of about because 80%. complete surgical resection was not able to perform due to the tumor extension. Furthermore a tumor debulking could not be Figure 2 MRI scan with skull base destruction by a large mass performed without extensive damage including facial palsy and hearing loss. infiltrating the right mastoid bone and the dura. Ann Otolaryngol Rhinol 2(5): 1039 (2015) 2/4 Küstermeyer et al. (2015) Email: Central

DISCUSSION It was Zuckerberg et al., Otalgia and hearing loss are most common symptoms in exclusively during the childhood who and first youth, defined and has the features term ENT practice. Using an otomicroscope allows the differentiation “Kaposiformhemangioendothelioma” in 1993. It occurs nearly between external otitis and acute otitis media, and shows edema ranging from those of a benign well-differentiated hemangioma and erythema of the tympanic membrane or middle ear effusion. resembling to capillary hemangioma and Kaposi , hemangio endotheliomas are often associated with consumption response by elevated leukocyte counts in peripheral blood to those of an an aplastic angiosarcoma [20]. Kaposiform andIn addition, increased laboratory c-reactive protein examinations [5]. Further detect examinations inflammatory are Merrit phenomenon [21], which could be excluded in the present required in cases of a suspected complication. In this case the coagulopathy and thrombocytopenia, the so called Kasabach- patient presented with symptoms of an acute otitis media with case. beginning mastoiditis, which were misleading and did not point In the year 1899 Nadoleczny already reported about the challenging differential diagnosis between endothelial tumors and of the temporal bone by means of precisely to theTumors insidious of the disease, temporal which bone was and finally particular diagnosed. in children are extremely rare. Their incidence varies from 1:5,000 through microscopic investigation [22]. In the present case correct 1:20,000, whereas most tumors are malignant with diagnosis is still challenging, although pathologists meanwhile being the most frequent tumor entity [7,8]. The occurrence are able to scrutinize surgically obtained material by using several of benign tumors of the temporal bone can only be estimated. special examinations, i.e. and molecular The incidence is expected as 1:40,000 through 1:200,000 [9]. biologic tests. Immunohistochemical markers expressed by cells Carcinomas in this area have an estimated yearly incidence of of endothelial lineage are CD31, CD34 and factor VIII, which are only 6 in 1 million people [10], and are known to be ten times more frequent than sarcoma. Although it is one of the rarest However, the of angiosarcoma is generally tumors of the temporal bone, sarcoma occurs more frequently in positive in tissue infiltrated by angiosarcoma [13,16]. described as poor, because of high rates of recurrence and children than in adults [8]. Angiosarcomas represent an amount [12,14,23]. To date, due to its rarity, no standardized recommendation for the treatment of intracranial angiosarcomas skullof less base than were 1% of nearly all sarcomas. exclusively The observedpercentage in of male intracranial patients is available [24]. The best prognosis is associated with complete withangiosarcomas an average is age less of than 24 years 2%. Reported [11]. The casesmajor involving part of bony the resection and histologically controlled clear margins. Adjuvant angiosarcomas occurs in the long of the extremities, the therapy with radiation and chemotherapy are discussed rips, pelvis and vertebra. Primary skull base angiosarcomas are controversially [25]. New therapy concepts are based on extremely rare, and to best of our knowledge less than 20 cases monoclonal antibodies, i.e. inhibitors of vascular endothelial have been reported in the literature [12,13]. growth factor, and are focused to improve the treatment outcome of angiosarcoma [26]. Risk factors for extra-cranial angiosarcomas are prior , arsenic exposure, long-standing lymph- Diagnostical methods of rare pathologies require an edema, and history of previous trauma [14]. However, currently interdisciplinary teamwork. Today’s technical possibilities in primary skull base angiosarcoma no risk factors are known allow an easy exchange of knowledge all around the world to [15]. characterize diseases precisely. This is crucial for a targeted and effective treatment. A variety of terms are used to describe this tumor beside angiosarcoma: hemangioendothelioma, hemangiosarcoma, ACKNOWLEDGEMENTS malignant angioendothelioma, and lymphangiosarcoma [14]. We are thankful to Dr. Rieder and Colleagues, Hanover, Frequent clinical symptoms are otalgia, hearing loss, tinnitus and a swelling in the temporal region, like in the present case Germany,REFERENCES for providing the CT image shown in (Figure 1). found i.e. in acute otitis media or in acute mastoiditis. 1. [11,13,16,17]. 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Cite this article Küstermeyer J, Ostertag H, Hattingen J, Welkoborsky HJ (2015) Tumor of the Temporal Bone Mimicking Acute Mastoiditis. Ann Otolaryngol Rhinol 2(5): 1039.

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