gms | German Medical Science

ESBS 2005: Skull Base Surgery: An Interdisciplinary Challenge
7th Congress of the European Skull Base Society held in association with
the 13th Congress of the German Society of Skull Base Surgery

18. - 21.05.2005, Fulda, Germany

The hemangiopericytoma – a rare head and neck tumor

Meeting Contribution

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  • Sven Koscielny - HNO-Klinik der Friedrich-Schiller-Universität, Jena, Deutschland

ESBS 2005: Skull Base Surgery: An Interdisciplinary Challenge. 7th Congress of the European Skull Base Society held in association with the 13th Congress of the German Society of Skull Base Surgery. Fulda, 18.-21.05.2005. Düsseldorf: German Medical Science GMS Publishing House; 2009. Doc05esbs62

doi: 10.3205/05esbs62, urn:nbn:de:0183-05esbs624

Published: January 27, 2009

© 2009 Koscielny.
This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by-nc-nd/3.0/deed.en). You are free: to Share – to copy, distribute and transmit the work, provided the original author and source are credited.


Outline

Text

Introduction

The hemangiopericytoma is a very rare tumor in the head and neck region. The first description of this tumor goes back to Scout and Murray in 1942 [10]. The tumor takes its origin from the pericapillary pericytic cells, which were described first by Carpenter in 1923 [10]. On histopathologic examination the tumor is characterized by a proliferation of oval and spindle-shaped pericytic cells. The literature distinguishes between a benign and a malignant form. Signs for a malignant transformation are an increased mitotic activity, a higher cell density, an appearance of undifferentiated cells as well as necrotic and hemorrhagic zones in the tumor tissue [3]. Clinically a malignant hemangiopericytoma is characterized by haematogenic metastases, typically to the lung, the liver and the skeleton system [1], [3]. On first diagnosis it is generally difficult to distinguish between benign and malignant forms. This frequently becomes evident only during the further course of the disease [3]. Comparing larger clinical observations the frequency of occurrence in the head and neck is estimated between 16% [8] and 33% [3] of all hemangiopericytomas occuring in various localisation’s. The most frequent locations are the lower and the upper limbs as well as the brain. Over all approximately 200 cases of hemangiopericytomas only were mentioned in literature over the last 30 years partly as mere case reports. This was the reason to review the patients with this diagnosis treated in our department with regard to our therapeutical strategy and the clinical courses.

Material and method

Inpatient and outpatient medical records from 1970 to 2001 were evaluated retrospectively.

The medical histories of the four patients we found having been treated because of a hemangiopericytoma are presented below.

Patient 1

A 57-year-old lady presented 1976 for the first time in our department. She complained of recurrent nose-bleeding for four weeks from the left side of the nose an a left sided nasal congestion. On clinical examination a tumor was found filling the whole of the left nasal cavity. The occipito-mental x-ray showed a complete occlusion of the left paranasal sinuses. In a conventional tomography of the paranasal sinuses destruction of the maxillary bone was seen. A biopsy was obtained. The pathohistological examination revealed a hemangiopericytoma. Treatment was an operation via lateral rhinotomy. During surgery the tumor was found filling the entire left nasal cavity and originating from the left maxillary sinus. The hemangiopericytoma had a high proliferation tendency. It was decided to refrain from a postoperative irradiation. The patient remained in our outpatients dispensaries. She died as consequences of a heart attack without signs of tumor recurrences in 1992.

Patient 2

A 44-years-old men was admitted in 1985 because of recurrent swelling of the right upper eyelid. At the time of admission he showed a latero-caudal displacement and protrusion of the right eye and complained of double images. The visus of the right eye was not impaired. Clinical investigation showed a livid tumor in the right nose. The conventional tomography of the paranasal sinuses (no CT-scan available at this time) showed a tumor of the right paranasal sinuses, which infiltrated the skull base around the posterior wall of the frontal sinus and the roof of the ethmoid sinus. The tumor had also invaded the orbita. A surgical approach was chosen for therapy including a resection of the right upper jaw and exenteration of the right orbita. During surgery a broad infiltration of the orbita and the skull base with an infiltration of dura mater on an area of 4x5 cm and an endocranial tumorinfiltration were found. After interim closure of the dura mater the endocranial part of the tumor was neurosurgically resected in a second operation. The pathohistological investigation of both tumor parts showed a hemangiopericytoma with an aggressive and bone-destructive growth. Radiotherapy was given in a dose of 60 Gy postoperatively. In 1999 an MRI showed an endocranial tumor in the opposite frontal lobe. This recurrence was treated neurosurgically again in January 2000 and postoperatively irradiated with 54 Gy. By the end of 2001 the patient was in a good general condition without signs of a tumor recurrences.

Patient 3

A 64-year-old women presented in our outpatient department with a three years history of a pulse synchronous tinnitus on the left ear without any hearing loss or vertigo in 1992. Microscopy of the left ear showed a pulsating reddish tumor behind the intact eardrum. Under suspicion of a glomustumor an MRI was initiated. It showed a 2x1x1 cm tumor in the hypotympanon with contact to the bulbus venae jugularis without endocranial infiltration. The supplying vessels originating from the maxillary and occipital arteries were selectively embolised. Thereafter the tumor was surgically resected. The tumor was 1 cm in diameter and situated in the glomus tympanicum. There was no infiltration to the bulbus venae jugularis. The pathologist found a hemangiopericytoma with a slight tendency of proliferation. Up to April 2002 the patient was still tumor free.

Patient 4

A 16-year-old women presented in our department in beginning of 2001. There was a state after three times repeated endonasal surgery of the maxillary sinus in another ENT-department within a year because of recurrent swelling of the left cheek for the past five years. The operation reports described bony-defects in the posterior and lateral part of the maxillary sinus, which were interpreted as result of an inflammation. The histpathological changes of the specimens had been interpreted as chronic-inflammatory changes. On examination we found spontaneous haemorrhages in the left upper and lower eyelid and a decrease sensitivity of the infraorbital nerve on this side. The CT- and MRI-scans showed a tumor, which had destroyed the bone of the maxillary sinus and infiltrated the lateral lower parts of the orbita (Figure 1 [Fig. 1], Figure 2 [Fig. 2], Figure 3 [Fig. 3]). After comprehensive discussion with the young patient and their parents and balancing the risks it was decided to resect the upper left with the option of an exenteratio orbitae. During surgery we found a broad infiltration of the orbital fat, and therefore the orbita was exenterated. Because of the infiltrating tumor growth an postoperative radiotherapy was discussed with the radiologist, the patient and her parents. Since there is no clear data about the sensitivity of hemangiopericytomas to irradiation, and because of the young age of the patient it was agreed to watch and wait. In may 2004 the patient was well and alive without signs of a recurrence.

Discussion

The diagnosis of hemangiopericytoma represents a clear rarity in the range of head and neck tumors. In our department we have treated a total of four patients with this diagnosis during the last 30 years. The average age was 45 years (16–64 years). The sex distribution shows a predominance of women (3 women against 1 men). We found in the described larger patient groups [1], [10] only on few hemangiopericatoma in the paranasal sinuses. The corresponding part of the most recent German manual of ENT-diseases by Ganzer et al. [5] speaks likewise of the very rare appearance of these tumor in the paranasal sinuses.

We could not find a description of a hemangiopericytoma of the middle ear in our literature search. Also in the very comprehensive essay on middle ears tumors by Draf [3] in the current German manual of ENT-diseases no hemangiopericytoma of the middle ear is described. The case described above therefore represents to our knowledge the first case of this localization in literature.

The distinction between benign and malignant forms in older literature [1], [2] was founded on the clinical course of the disease (occurrence of metastases, recurrences). A more recent investigation tried to define the differences between the two forms using a molecularbiological marker (Ki-67) to calculate a proliferation index [6].

As therapeutical standard a radical resection is favored. The value of a post-operative irradiation is discussed very differently in literature. Whilst Draf and Berghaus [6], Ganzer et al. [6], Enzinger [4] and others [5], [9] see an adjuvant radiotherapy very critically, Borg et al. [1] reported better results after post-operative irradiation. However there have been no other authors in favor of this view so far. In our reviewed collective radiotherapy was applied only to the second patient with a widespread tumor with endocranial infiltration. This patient was the only, who developed a metastatic recurrences in the opposite frontal lobe, which again was treated with a combination of surgery and post-operative irradiation.

All our other patients were treated surgically alone, as recommended by Draf and Berghaus [2] and Ganzer et al. [5]. Despite of a high proliferation tendency as described in the histopathologic reports in our cases 1 and 3 the patients are still free of recurrence without post-operative irradiation.

To find the right diagnosis of hemangiopericytoma is not always simple. However it is essential for an appropriate treatment. As shown in our case 4 it should always be paid attention to the consistency of clinical picture, the local findings during surgery and the results of pathohistological investigations. If the are inconsistencies another experienced pathologist should be involved for a second opinion. A reevaluation of the histologic slides of our patient 4, that were misinterpreted as chronic sinusitis, revealed structures of the missed tumor. Therefore we think, it is the task of the clinical physician work on this synopsis of all findings and in case of discrapancies to initiate further diagnostic measures to achieve a clear and consistant picture.

Conclusion

Due to the heterogeneous facts in literature, we see at present the best therapy of the hemangiopericytoma in an operation with onco-surgical standards. Only in selected individual cases (e.g. an endocranial infiltration or broad infiltration of adjacent structures) we would discuss with the patient the possibility of an adjuvant irradiation.


References

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