A rare presentation of myxofibrosarcoma as a Pancoast tumor: a case report
© The Author(s). 2017
Received: 6 October 2016
Accepted: 25 January 2017
Published: 7 March 2017
Myxofibrosarcoma is an aggressive soft tissue neoplasm, classified as a variant of malignant fibrous histiocytoma. Most often, it occurs in middle to late adult life peaking in the seventh decade and involving the lower extremities (77%), trunk (12%), and retroperitoneum or mediastinum (8%). We report the first case of thoracic myxofibrosarcoma presenting as a Pancoast tumor.
A 48-year-old non-tobacco smoking African-American man presented with a slow-growing mass in his neck along with 11 kg weight loss over 9 months. A review of his systems was positive for hoarseness and lowgrade intermittent fever without any shortness of breath or cough. A physical examination revealed a mass on the left side of his neck superior to his sternoclavicular joint measuring 3 × 3 × 1 cm. He had ptosis and miosis of his left eye. His breath sounds were decreased and coarse at the left apex. A neurological examination revealed 3/5 strength in his left upper arm. The remainder of the physical examination was unremarkable.
Ultrasound of his neck showed an ill-defined heterogeneous mass lateral to his left thyroid lobe. A computed tomography scan of his chest showed a large multiloculated pleural-based mass in his left lung surrounding the adjacent neurovascular structures. A percutaneous biopsy was non-diagnostic. Subsequently, he underwent a left thoracotomy with biopsy. The mass extended from his anterior mediastinum medially at the level of the pulmonary trunk, superiorly into the superior sulcus and posteriorly into his chest wall. Surgical pathology confirmed the diagnosis of myxofibrosarcoma.
Here we present a case of Pancoast tumor with myxofibrosarcoma as the underlying etiology. Pancoast syndrome generally entails an infiltrating lesion in the superior sulcus presenting with upper extremity pain, atrophy of the hand muscles, and Horner’s syndrome. The differential diagnosis of Pancoast syndrome includes inflammatory and infectious etiologies, as well as neoplasms of benign and malignant nature. Of the neoplasms implicated, the most common are non-small cell lung carcinomas; myxofibrosarcoma presenting as a Pancoast tumor has not been reported in the literature.
KeywordsCase report Myxofibrosarcoma Pancoast syndrome Horner’s syndrome Malignant fibrous histiocytoma
Myxofibrosarcoma (MFS) is an aggressive soft tissue neoplasm classified as a variant of malignant fibrous histiocytoma (MFH). On histologic examination it is composed of both fibroblastic and histiocytic elements. Most cases occur in middle-to-late adult life with a peak incidence in the seventh decade [1, 2]. They are predominantly encountered in the lower extremities (77%), trunk (12%), and retroperitoneum or mediastinum (8%) [1–3]. To the best of our knowledge only 19 cases of head and neck MFSs have been described in the literature, making it exceedingly rare . For these sarcomas, the best approach to treatment is complete surgical resection . Of all case presentations, none have had Pancoast syndrome as the initial manifestation. We are reporting the first case of MFS presenting as a Pancoast tumor.
Pancoast syndrome involves an infiltrating mass at the apex of the lung [4, 5]. Clinical manifestation is related to lesion location in the superior sulcus, with ipsilateral shoulder and arm pain, and weakness and atrophy of the ipsilateral hand muscles all due to brachial plexus compression or invasion. Commonly, Pancoast tumors also exhibit concurrent Horner’s syndrome along with other signs and symptoms of malignancy.
The majority of superior sulcus tumors are non-small cell lung carcinomas (NSCLCs) [5, 6]. Other etiologies less common for Pancoast syndrome include inflammatory, infectious, and neoplastic entities [4–6]. The treatment modalities for Pancoast tumor are determined by the specific nature of the neoplastic process in question. Surgical intervention is indicated in the majority of these instances. The presence of Horner’s syndrome and advanced disease are associated with a dire prognosis [4, 6].
Head and neck presentation of MFS is extremely rare. To the best of our knowledge, of the 19 cases of head and neck MFS reported in the literature, our case is the first presenting as a Pancoast tumor. Pancoast syndrome entails superior sulcus infiltration presenting with upper extremity pain and atrophy of the hand muscles, commonly with concurrent Horner’s syndrome [5, 6]. The differential diagnosis of a Pancoast syndrome includes inflammatory etiologies such as sarcoidosis and granulomatosis with polyangiitis, infectious etiologies such as tuberculosis and aspergillosis, and neoplasms of benign and malignant nature [5, 6]. Of the neoplasms implicated, the most common are NSCLCs; MFS presenting as a Pancoast tumor has not been reported in the literature .
MFS is characterized by nodular growth of spindle-shaped or stellate-shaped fibroblastic cells in a myxoid matrix [2, 9]. This entity has been divided into four grades pertaining to cellularity, atypia, and extent of myxoid component [1–3, 9]. High-grade MFS (HGMFS) demonstrates a dominance of patternless histiocyte-like cells with pleomorphism in a minimal myxoid matrix . Characteristic features differentiating this grade of lesion include areas of necrosis with lymphocytic infiltration, numerous and atypical mitotic figures, multinucleated giant cells, and extravasation of erythrocytes [2, 3, 9]. HGMFS lesions are frequently encountered in intramuscular and subfascial anatomy as opposed to the superficial (subcutaneous or dermal) manifestations of lower-grade lesions [2, 3, 9]. The differential diagnosis for HGMFS includes lesions of lower grade, nodular fasciitis, myxoma, extraskeletal myxoid chondrosarcoma, and pulmonary artery sarcomas [2, 3, 7, 10]. The characteristic features of our case presentation favored HGFMS as a diagnosis over other etiologies and lesions of lower grade.
Mentzel, Angervall, Merck, and their colleagues have previously described the relation between histologic grade of MFS and rate of local recurrence [2, 9]. The local recurrence of HGMFS had been documented as 55%, 64%, and 61% by Mentzel, Angervall, and Merck, respectively [2, 9]. The patient in our case report did not demonstrate any metastatic lesions. Mentzel, Angervall, and Merck had similarly described metastatic rates as 29%, 27%, and 38%, respectively [2, 9]. HGMFS has a less favorable outcome when compared to lower grades, with a mean survival of 2 months without intervention and extension to 10 months with tumor resection [11, 12]. As such, complete surgical resection is the mainstay treatment for MFS. Unfortunately, there is little literature regarding combination radiochemotherapy for cases of MFS that have suboptimal or no surgical excision [1, 13]. Although the tumor was evidently a suboptimal candidate for complete surgical resection, less invasive modalities of histological diagnosis were not employable. Thus, a left thoracotomy was opted for with the goal of definitive tissue diagnosis and resection of safely removable tumor mass. Unfortunately, an insufficient margin of resection was the result due to the extensive involvement of critical structures. Mediastinal excision of the mass measuring 6 × 6 cm was performed with partial decortication. More studies are necessary to investigate the efficacy of adjuvant radiochemotherapy for head and neck MFS, with a focus on cases where surgical management is difficult to attain. Although this entity is difficult to treat, follow-up entails assessing symptom progression and serial imaging [1, 9, 13].
Our patient had requested transfer of care to an oncology center with more proximity to his home address. They had begun treatment with high-dose ifosfamide. The first cycle of treatment was well tolerated, resulting in an apparent decrease in tumor size. He had returned for a second cycle of ifosfamide with an initial dose of high-intensity radiation with the high-dose spatially-fractionated radiation (GRID) approach followed by additional doses with conventional radiation.
In conclusion, MFS is a relatively uncommon malignancy and has not been described as a Pancoast tumor in the medical literature. Surgical resection may result in insufficient margins when extensive invasion within the superior sulcus and critical structures of the thorax is involved. Given that this management is the mainstay for MFS, more research is necessary with regards to adjuvant therapies that can improve prognosis.
Malignant fibrous histiocytoma
Non-small cell lung carcinoma
The authors would like to acknowledge the Research Open Access Publishing (ROAAP) Fund of the University of Illinois at Chicago for financial support towards the open access publishing fee for this article.
Availability of data and materials
Data sharing is not applicable to this article as no datasets were generated or analyzed during the current study.
VJ, AY, and EGC analyzed and interpreted the patient data regarding the disease, and conducted patient care. VJ collected patient data, described it in the case report with literature review. AY, ZH, AA, and EGC performed the literature review and made significant contributions to the writing of the manuscript. All authors read and approved the final manuscript.
The authors declare that they have no competing interests.
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Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
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- Dell’Aversana Orabona G, Iaconetta G, Abbate V, Piombino P, Romano A, Maglitto F, Salzano G, Califano L. Head and neck myxofibrosarcoma: a case report and review of the literature. J Med Case Reports. 2014;8:468.View ArticleGoogle Scholar
- Weiss SW, Goldblum JR. Fibrosarcoma. In: Weiss SW, Goldblum JR, editors. Enzinger and Weiss’s Soft Tissue Tumors. 4th ed. St Louis: Mosby; 2001. p. 422–5.Google Scholar
- Kindblom LG, Merck C, Angervall L. The ultrastructure of myxofibrosarcoma: A study of 11 cases. Virchows Arch A Pathol Anat Histol. 1979;381(2):121–39.View ArticlePubMedGoogle Scholar
- Arcasoy SM, Jett JR. Superior Pulmonary Sulcus Tumors and Pancoast’s Syndrome. N Engl J Med. 1997;337(19):1370–6.View ArticlePubMedGoogle Scholar
- Foroulis CN, Zarogoulidis P, Darwiche K, Katsikogiannis N, Machairiotis N, Karapantzos I, Tsakiridis K, Huang H, Zarogoulidis K. Superior sulcus (Pancoast) tumors: current evidence on diagnosis and radical treatment. J Thorac Dis. 2013;5(S4):S342–58.PubMedPubMed CentralGoogle Scholar
- Parissis J, Young V. Treatment of Pancoast tumors from the surgeons prospective: re-appraisal of the anterior-manubrial sternal approach. J Cardiothorac Surg. 2010;5:102.View ArticlePubMedPubMed CentralGoogle Scholar
- Elphinstone RH, Spector RG. Sarcoma of the Pulmonary Artery. Thorax. 1959;14:333–40.View ArticlePubMedPubMed CentralGoogle Scholar
- Varriale P, Chryssos B. Pulmonary Artery Sarcoma: Another Cause of Sudden Death. Clin Cardiol. 1991;14:160–4.View ArticlePubMedGoogle Scholar
- Mentzel T, Calonje E, Wadden C, Camplejohn RS, Beham A, Smith MA, Fletcher CD. Myxofibrosarcoma. Clinicopathological analysis of 75 cases with emphasis on the low-grade variant. Am J Surg Pathol. 1996;20(4):391–405.View ArticlePubMedGoogle Scholar
- Green Jr JR, Crevasse LE, Shanklin DR. Fibromyxosarcoma of the pulmonary artery. Am J Cardiol. 1964;13(4):547–52.View ArticlePubMedGoogle Scholar
- Kaplinksy EJ, Favaloro RR, Pombo G, Perrone SV, Vigliano CA, Schnidt JL, Boughen RP. Primary pulmonary artery sarcoma resembling chronic thromboembolic pulmonary disease. Eur Respir J. 2000;16:1202–4.View ArticleGoogle Scholar
- Furest I, Marin M, Escribano P, Gomez AA, Cortina J, Blanquer R. Intimal Sarcoma of the Pulmonary Artery: a Rare Cause of Pulmonary Hypertension. Arch Bronconeumol. 2006;42(3):148–50.View ArticlePubMedGoogle Scholar
- Cante D, Franco P, Sciacero P, Girelli GF, Casanova Borca V, Pasquino M, Tofani S, Bombaci S, Migliaccio F, Marra A, Numico G, La Porta MR, Ricardi U. Combined chemoradiation for head and neck region myxofibrosarcoma of the maxillary sinus. Tumori. 2013;99(2):e80–3.PubMedGoogle Scholar