Giant cervico-mediastinal solitary fibrous tumor
Alberto Schreiber, Davide Lombardi, Francesco Missale, Davide Farina, Luisa Lorenzi & Piero Nicolai European Archives of Oto-RhinoLaryngology and Head & Neck ISSN 0937-4477 Eur Arch Otorhinolaryngol DOI 10.1007/s00405-014-2943-4
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Author's personal copy Eur Arch Otorhinolaryngol DOI 10.1007/s00405-014-2943-4
Case Report
Giant cervico‑mediastinal solitary fibrous tumor Alberto Schreiber · Davide Lombardi · Francesco Missale · Davide Farina · Luisa Lorenzi · Piero Nicolai
Received: 19 November 2013 / Accepted: 6 February 2014 © Springer-Verlag Berlin Heidelberg 2014
Abstract Objectives Extra-pleural solitary fibrous tumor (ESFT) is an uncommon mesenchymal neoplasm that is anatomically ubiquitous and may be found anywhere in the body, including the head and neck region. It is usually asymptomatic and presents as a slowly growing painless mass. Only three cases of retropharyngeal ESFT have been reported in the literature. Methods and results A 54-year-old female affected by a cervicomediastinal mass complained of progressive dysphonia, pharyngeal foreign body sensation, and mild dyspnea. CT and MR showed a huge retropharyngoesophageal lesion extending to the upper posterior mediastinum. The tumor, despite its caudal extension, was completely removed with a pure cervicotomic approach; histology was consistent with ESFT. Conclusions Histopathology and immunohistochemistry are crucial in the diagnosis of solitary fibrous tumor. Radical excision after primary treatment is the most important indicator of prognosis, and long-term clinical follow-up is recommended due to the possibility of recurrence and/or malignant transformation.
A. Schreiber (*) · D. Lombardi · F. Missale · P. Nicolai Department of Otorhinolaryngology, University of Brescia Piazza, Spedali Civili 1, 25123 Brescia, Italy e-mail:
[email protected] D. Farina Department of Radiology, University of Brescia Piazza, Spedali Civili 1, 25123 Brescia, Italy L. Lorenzi Department of Pathology, University of Brescia Piazza, Spedali Civili 1, 25123 Brescia, Italy
Keywords Solitary · Fibrous tumor · Mediastinal tumors · Retroesophageal lesion · Retropharyngeal lesion · Hemangiopericytoma
Introduction Under the umbrella term of solitary fibrous tumor (SFT), there is a heterogeneous group of rare spindle-cell tumors that comprises benign and malignant neoplasms with an unclear cellular origin. After its first recognition in the pleura and other serosal surfaces, it was rapidly realized that SFTs could also be observed in extra-pleural sites. Extra-pleural solitary fibrous tumors (ESFTs) have been described almost everywhere in the body including soft tissues and visceral organs [1]. Clinically, ESFT usually presents as a well-defined, slow-growing, and painless mass. It is frequently asymptomatic, but may occasionally lead to compression symptoms, especially when a large size is reached. The behavior of SFT is unpredictable. About 10 % behave aggressively, and local or distant recurrence can occur even many years after primary resection [2]. The treatment of choice for localized SFTs is surgery with free margins [3]. Chemotherapy and radiation therapy have not been shown to have a significant role as adjuvant treatment, but radiation can be considered in cases of SFTs with malignant features, especially in the presence of incomplete excision. Even though SFT can be found in the head and neck region, to the best of our knowledge, only three cases with retropharyngeal localization have been described in the literature [4–6], one of which extended from the retropharyngeal space (RPS) to the posterior mediastinum [6].
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Fig. 1 CT scan. A large retropharyngeal mass shows mixed basal density (a) and nonhomogenous contrast uptake (b–d). The lesion dislocates the azygos vein (white arrow in b), esophagus (black arrows in b), and neck vessels (arrows in a) without signs of infiltration. A network of hypertrophic feeders is seen on the upper part of the mass (arrows in c). The sagittal plane (d) fully depicts the vertical extent into the mediastinum
Case report Diagnostic workup A 54-year-old woman was first seen at our outpatient clinic for a slowly progressive dysphagia lasting 2 years, associated during the last months with a pharyngeal foreign body sensation and mild dyspnea, especially in the supine position. The patient never smoked and her medical history was unremarkable. Several endoscopic evaluations performed at other institutions had ruled out any expansile lesion of the upper aerodigestive tract; no cranial nerve dysfunction was observed and a diagnosis of laryngo-pharyngeal reflux was initially made. Due to persistent complaints, the patient underwent ultrasound of the neck, which showed a hypo-echoic mass with well-defined margins localized posteriorly to the thyroid gland and trachea, with small cystic spaces and a vascularized aspect upon color Doppler evaluation. CT (Fig. 1) assessed the space of origin of the lesion (i.e., retropharyngeal space) and its vertical extent, from the lower
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suprahyoid neck to the carina. Well-defined borders and dislocation of adjacent structures (esophagus, vascular bundles, azygos vein, prevertebral muscles) without any signs of infiltration suggested a slow-growing tumor with limited aggressiveness. The lesion was slightly hypodense to the muscles, did not contain fat tissue or calcifications, and was overall intense with non-homogeneous contrast enhancement and small feeding vessels at its periphery. Finally, MR was performed (Fig. 2a–c) to confirm that the spine and medulla were not involved and to further characterize the tumor. The T2 signal was brighter than fat tissue, and the rich network of newly formed vessels within and around the lesion was clearly demonstrated; furthermore, diffusion-weighted imaging (DWI) showed no signs of restriction of random motion of water molecules, thus corroborating the suggestion of a non-aggressive tumor, possibly of benign nature. Bronchoscopy with transbronchial aspiration biopsy was non-diagnostic, and showed extrinsic compression of the posterior wall of the trachea with no signs of infiltration. Endoscopic evaluation of the esophagus showed
Author's personal copy Eur Arch Otorhinolaryngol Fig. 2 MR (a–c) and digital subtraction angiography (d). Vascular feeders are seen as hypointense dots and serpiginous lines (arrows in a and c) at the periphery of the T2-hyperintense mass. High signal on DWI scan (b) indicates no restriction of random motion of water molecules within the mass, thus suggesting a benign nature. Selective catheterization of the hypertrophic branch of the external carotid artery (d) supplying the lesion shows a network of arterial vessels feeding the mass. Embolization with polyvinyl alcohol particles obtained an estimated 70 % devascularization
compression and dislocation of its cervical portion with normal mucosa. Treatment Due to the hypervascularized nature of the lesion, 48 h before surgery the patient was submitted to angiographic embolization (Fig. 2d) of the largest vascular feeders, arising from the external carotid artery, obtaining an estimated 70 % vascular supply reduction. It was planned to remove the lesion through a combined transcervical and posterolateral thoracotomic approach. The tumor appeared as a soft, grayish, well-defined lesion. The internal jugular veins and common carotid arteries were identified and dissected off the lesion. The parathyroid glands and recurrent nerves were anteriorly dislocated by the mass lying posterior to the esophagus. The superior thyroid pedicles were ligated bilaterally, and the prevertebral space was reached without any sign consistent with infiltration. Gentle traction on the retropharyngo-esophageal portion of the tumor allowed its complete isolation into the neck. The cervical portion of
the lesion was finally cut and removed to better expose the remaining portion into the posterior mediastinum. Blunt dissection and progressive gentle traction of the mediastinal part of the lesion as well as the absence of adhesions and significant mediastinal vascular supply allowed its complete removal without thoracotomy. A left recurrent nerve palsy was identified at laryngoscopy performed immediately after extubation. Histopathology Microscopically, the lesion was composed of spindle cells with abundant eosinophilic cytoplasm and oval nuclei showing very low mitotic activity (Fig. 3b). The tumor cells were arranged in small bundles and dispersed in abundant stroma with areas of hyalinosis, fibrosis, and edematous and focal cystic degeneration. The lesion was intersected by numerous vessels with a hemangiopericytoma pattern (Fig. 3a). At immunohistochemistry (IHC), tumor cells showed diffuse and intense expression of CD34 and Bcl2 (Fig. 3b–c), whereas CD31, smooth muscle actin,
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Fig. 3 Hematoxylin–eosin (h–e) stain showing a solid proliferation composed of medium-sized ovoid and spindle cells with bland nuclei and occasional nucleoli, intersected by numerous vessels with a hemangiopericytoma-like pattern [a [×10], b (×40)]. Immunohisto-
chemistry for CD34 (magnification ×20) showed strong and intense membranous positivity for tumor cells with endothelial cells as internal positive control (b). Tumor cells also showed cytoplasmic expression of Bcl2 (×20) (c)
muscle-specific actin, podoplanin, cytokeratin and S-100 protein stained negative. The proliferation index, evaluated by MIB-1/Ki67, was 5 %. The definitive histology was therefore consistent with SFT.
of this tumor has been controversial. Although Stout and Murray [9] proposed mesothelial cells as the source of the so-called localized mesothelioma, it is now known that this tumor is quite ubiquitous, having been identified in many extra-pleural sites wherever mesenchymal tissue is located [10, 11]. More recently, it has been reported that SFTs may originate from CD34-positive, interstitial dendritic cells of connective tissues [12]. This hypothesis is consistent with the fact that ESFT can be observed at any location: 40 % are found in subcutaneous tissues, while others arise in deep soft tissue of the extremities or in the head and neck region, as well as many other locations [1, 5, 13–16]. Since the histologic diagnostic criteria for SFT and hemangiopericytoma almost coincide and there is no clear distinction between these two entities, pathologists have been gradually abandoning the term “hemangiopericytoma” in favor of SFT; indeed, in the fourth edition of the World Health Organization Classification of Tumors of Soft tissue and Bone [1], the term “hemangiopericytoma” was definitively defined as “obsolete” and SFT was classified as “typical” or “malignant” according to the number of mitosis,
Follow‑up Six days later the patient was discharged. Two months later left arytenoid motility appeared completely restored. MR at 6 and 20 months after surgery confirmed no evidence of residual or recurrent disease.
Discussion Solitary fibrous tumor (SFT) is an uncommon spindle-cell neoplasm first described by Wagner in 1870 [7]. Klemper and Rabin interpreted SFT as a form of localized mesothelioma that was different from diffuse mesothelioma due to the absence of correlation with asbestos exposure and good prognosis [8]. For a long time, the histogenesis
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cellular atypia, presence of necrosis, and hypercellularity. Microscopically, SFTs are characterized by a combination of hypocellular and hypercellular areas divided by bands of hyalinized collagen and thin-walled hemangiopericytomalike vessels. Tumor cells are ovoid to spindle-shaped with limited pale cytoplasm, ill-defined margins, and dispersed chromatin within vesicular nuclei. Myxoid change, fibrosis, and interstitial mast cells are commonly observed. Mitoses are generally scarce, rarely exceeding three mitoses per ten HPF. Malignant SFTs are usually hypercellular lesions, showing increased mitotic rate (>4 mitoses for 10 HPF), cytological atypia, tumor necrosis, and/or infiltrative margins; among these parameters, the number of mitoses seems to have the most relevant prognostic role [1, 17]. Immunohistochemically, SFTs commonly express CD34 (80–90 % of cases) and CD99 (70 %). Bcl-2 (30 %), epithelial membrane antigen (30 %) and smooth muscle actin (20 %) may also be expressed. Conversely, SFTs are usually negative for S-100 protein, desmin, and cytokeratins [1, 2]. SFT occurs with equal frequency in both sex and develops between the third and seventh decades of life; it is usually of a benign nature, with the malignant form occurring in 12–33 % of cases [1, 18, 19]. The clinical behavior of the tumor is unpredictable. Fifty percent of patients are asymptomatic and the tumor is discovered incidentally after radiologic imaging performed for unrelated causes. Signs and symptoms are usually non-specific and associated with larger tumors and occur more frequently in malignant subtypes. Symptoms are a result of either local compression/ invasion of adjacent structures (e.g., dyspnea, angina, and/ or cough) or paraneoplastic syndromes (e.g., hypertrophic osteoarthropathy, hypoglycemia, weight loss) [17]. Although identification at imaging of ESFT is quite difficult due to its rarity and potentially ubiquitous location, some CT and MR findings reported in the literature, and also found in the present case, may evoke this disease entity. In their retrospective analysis of CT findings in 18 SFTs, Papathanassiou et al. found in 11 of 18 cases on basal scans the same mixed pattern we observed, with iso- and hypodense areas. In the same series, the MR T2 signal was reported to be hyperintense or heterogeneous in the majority of cases; hypointensities scattered within the lesions are interpreted as areas of increased collagen content [20]. However, the most distinctive features are probably represented by the flow voids [21] and by the intense, even if non-uniform, contrast enhancement. Though not pathognomonic, the combination of these signs restricts the differential diagnosis to hypervascular tumors, such as SFT, paraganglioma—which is, however, a more sitespecific lesion—or hemangioma which, in general, shows a brighter T2 signal than SFT. Juvenile angiofibroma may share similar MR findings, although the combination of sex and age of the patient and site of the lesion easily guide
the differential diagnosis. Although the role of FDG-PET in the diagnostic workup must be further defined, it could possibly predict or preoperatively rule out malignancy in an SFT and may reveal metastatic recurrence, an event rarely observed in these tumors. Large SFTs with increased FDG uptake have a greater likelihood for malignancy [22]. Only three previous cases of retropharyngeal ESFT have been described in the literature [4, 5] and one extending from the RPS to the mediastinum [6]. Casani et al. [4] reported the case of an 81-year-old male with a huge SFT of the RPS extending from the plane passing through the inferior edge of the mandible up to the cricoid cartilage. The lesion was easily dissected from the surrounding tissues and radically removed with a transcervical approach. A transoral excision via a soft palate split approach of a retropharyngeal SFT was described by El-Sayed et al. [5]. in a 62-year-old male. The lesion was found to extend from the nasopharynx to the hypopharynx. To facilitate transoral excision under direct visualization, the central mass was partially excised to debulk the tumor. The third case is reported by Witkin et al. [6] in a series of 14 patients affected by SFTs of the mediastinum. A 51-year-old man presented with an 18.5-cm retropharyngeal lesion causing neck enlargement and positional dyspnea; no details about the surgical procedure and follow-up were provided. Because of the malignant potential of SFTs and uncertain diagnosis by needle aspiration biopsy, surgical exploration is mandatory, even in asymptomatic patients and extensive resection with curative intent is the gold standard for benign SFTs. In the present case, the large size of the lesion, extension into critical areas, and its uncertain nature contributed to the need not only for a demanding preoperative diagnostic workup, but also for complex surgical planning. It was planned to start with a cervicotomy to isolate the most cranial part of the mass and to identify the main feeding vessels with a proper control of internal jugular veins and carotid arteries. With regard to the extension into the posterior mediastinum, a postero-lateral thoracotomic approach was considered to be ideal to completely remove the lesion and minimize the risk of damage to the superior mediastinum great vessels. Adjuvant postoperative chemotherapy, radiotherapy, or both have been used in malignant SFTs, although their efficacy has not been demonstrated [19, 22]. When the tumor cannot be removed surgically or when metastases occur, chemotherapy and/or radiotherapy can be proposed as palliative treatment [3]. In the case of benign SFTs, the recurrence rate is reported to be only 2 % after surgical excision and may be due to incomplete resection, unrecognized malignancy, or the growth of an unrelated second SFT. In case of malignant forms, approximately half of the patients
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can be cured, whereas the remainder often develop recurrences and metastases [17, 23, 24]. In conclusion, ESFTs are rare in the head and neck region and usually present as slow-growing asymptomatic mass. Although there are some suggestive radiologic findings, diagnosis is obtained by definitive histology encompassing both morphologic and immunohistochemical features. Surgery is the primary treatment and has to be modulated based on the tumor site and size. Long-term clinical follow-up is recommended for all patients with SFT because of its potentially unpredictable behavior that can lead to recurrences and/or malignant transformation. Acknowledgments All authors confirm that they have no conflicts of interest with any companies/organizations.
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