Ann Thorac Surg 1998;66:2098-2099
© 1998 The Society of Thoracic Surgeons
Case Reports
Primary desmoid tumor of the posterior mediastinum
Theodosios J. Dosios, MDa,
Dimitrios C. Angouras, MDa,
Dimosthenis G. Floros, MDb
a Second Department of Propedeutic Surgery, Division of Thoracic Surgery, Athens University Medical School, Athens, Greece
b Department of Pathology, Hellenic Air Force and V.A. General Hospital, Athens, Greece
Accepted for publication May 26, 1998.
Address reprint requests to Dr Dosios, 2, Chatzigianni Mexi Street, 115 28 Athens, Greece
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Abstract
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We describe a unique case of a de novo desmoid tumor of the posterior mediastinum in a 21-year-old nulligravida. The tumor recurred twice despite its histologically confirmed radical extirpations. Because of the aggressive local behavior of desmoid tumors and their unusual locations, which prevent wide excision margins, we favor the early consideration of adjuvant therapy.
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Introduction
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Desmoid tumors are infrequent soft-tissue neoplasms comprising less than 0.1% of all solid tumors [1, 2]. They originate principally from deep-seated fascial or musculoaponeurotic structures and chiefly affect the abdominal wall. The extra-abdominal desmoid tumors occur within the muscles of the shoulder and pelvic girdle, the thoracic wall, and other less frequent sites [1–5]. Intrathoracic desmoid tumors are rare, only 14 cases having been reported in the English language literature [4, 6]. We describe a case of a de novo desmoid tumor of the posterior mediastinum.
A 21-year-old white nulligravida with a 3-month history of progressively increasing pain in her left shoulder and upper arm was admitted to the Second Department of Propedeutic Surgery of Athens University Medical School. Her family and past medical history were unremarkable and she denied smoking, alcohol consumption, and drug usage. On physical examination, tenderness of her left supraclavicular fossa and shoulder was noted, as well as mild weakness of her left upper arm. Results of baseline laboratory tests were normal, but chest x-ray revealed an ovoid mass in the left apex. A computed tomographic scan (Fig 1) and magnetic resonance imaging scan showed it to be a homogeneous solid mass, abutting on the posterior portions of the first through third left ribs, without evidence of rib, vertebra, or nerve invasion.
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Fig 1. Chest computed tomographic scan demonstrating the mass in the region of the left apex. (1 indicates the tumor;  used to determine density of the lesion.)
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A left posterolateral thoracotomy was performed. A firm, well-circumscribed 5.5-cm mass was found, with intimate attachment to the posterior portions of the second and third ribs and to the second costovertebral joint. The mass was radically excised en block with segments of the first through fourth ribs, the left transverse process of T2 vertebra, and the adjacent portion of the thoracic sympathetic chain, which was enclosed by the tumor. Postoperatively the patient manifested Horner syndrome, but her recovery was otherwise uneventful and the presenting symptoms disappeared.
Microscopically, the mass consisted of elongated slender spindle cells of uniform appearance surrounded by abundant collagen. The cells were arranged in sweeping bundles and immunostained with monoclonal antibodies for vimentin, but not for keratin, desmin, smooth muscle antigen, and S-100 protein. The microscopic diagnosis of extra-abdominal desmoid tumor was made.
Nine months later, the patient’s symptoms returned. Radiologic examinations revealed a 4-cm recurrent tumor in the posterior mediastinum. A new radical resection of the tumor was performed, along with segments of the third through fifth ribs and the transverse processes of T3 and T4 vertebrae.
Three months later, the symptoms reappeared. A sensitive, 6-cm circumscribed mass at the base of the left neck was found, abutting on the C7 and T1 transverse processes. The magnetic resonance imaging scan (Fig 2) revealed the mass obstructing the left vertebral artery and compressing, but not invading, the left subclavian artery and vein and the internal jugular vein. The tumor was radically excised along with the transverse process of C7 vertebra, via a left cervical incision extended to the left parasternal region and accompanied by disarticulation of the respective sternoclavicular joint. The left vertebral artery and internal jugular vein were ligated and divided. The postoperative course was uneventful. In both recurrences, histologic examination showed a desmoid tumor with free excision margins.
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Fig 2. Magnetic resonance image of the neck and thoracic outlet showing the recurrent tumor and its relation to adjacent vascular structures.
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The patient’s middle and lower left neck and left superior mediastinum were subsequently irradiated with 3,960 cGy, and adjuvant therapy with tamoxifen (20 mg/day) was administered for 3 months. The patient remains without evidence of recurrence 20 months after the third operation.
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Comment
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Most intrathoracic desmoid tumors represent intrathoracic extensions of thoracic wall or neck tumors [6], and many develop in scar tissue of a previous thoracotomy site [4, 6] supporting the hypothesized etiologic relation between trauma and fibromatosis [7]. On the basis of radiologic and surgical findings, as well as the absence of history of thoracotomy or nonsurgical trauma, we con-sider the tumor in our patient a de novo neoplasm of the posterior mediastinum [6, 8].
Desmoid tumors are characterized by the discrepancy between a deceptively benign histologic appearance and an aggressive clinical behavior, mainly involving invasion of contiguous structures and local recurrence [1–3, 5, 7]. This especially applies to young fertile and middle-aged women; a tumor growth rate of 2 to 4 times faster has been found in this group of patients, as the tumor growth is directly related to endogenous estrogens [7]. The pronounced tendency toward local failure has prompted some authors to consider desmoid tumors low-grade soft-tissue sarcomas [3, 5]. Aggressive resection with wide margins is essential for the treatment of desmoid tumors [1–3], but the location of the tumor in some patients limits margins, and therefore increases the attractiveness of adding adjuvant therapy, particularly radiation therapy [2].
Several reports have demonstrated efficacy of external beam radiation [4] or interstitial brachytherapy [5] in decreasing the local recurrence rate, but controversy remains concerning the indications for, and the timing of, adjuvant radiation therapy [2, 3]. Similarly, the role of various pharmacologic approaches, including estrogen blockade, prostaglandin inhibition, and cyclic adenosine monophosphate modulation, remains ill defined [1–3].
Despite the controversial reports on adjuvant therapy for desmoid tumors, this case report demonstrates that an unusual location of a desmoid tumor, which prevents wide excision margins, favors the early consideration of adding adjuvant therapy, especially in premenopausal women.
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Acknowledgments
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Dr Juan Rosai, Chairman, James Ewing Alumni Chair in Pathology, Memorial Sloan-Kettering Cancer Center reviewed the histologic sections of the first recurrent tumor.
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References
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Abstract
Introduction
