Ann Thorac Surg 2001;71:1698-1700
© 2001 The Society of Thoracic Surgeons
Case report
Intrathoracic desmoid tumor mimicking primary lung neoplasm
Mobeen Iqbal, MDa,
Leonard J. Rossoff, MDa,
Leonard Kahn, MDa,
Rudy P. Lackner, MDa
a Division of Cardiothoracic Surgery, Long Island Jewish Medical Center, New Hyde Park, New York, USA
Accepted for publication June 3, 2000.
Address reprint requests to Dr Rossoff, Division of Pulmonary and Critical Care Medicine, Long Island Jewish Medical Center, The Long Island Campus of the Albert Einstein College of Medicine, Room C-20, 270-05 76 Ave, New Hyde Park, NY 11042
e-mail: lackner{at}lij.edu
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Abstract
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Most reported thoracic desmoid tumors originate from the chest wall. However, intrathoracic desmoid tumors are rare. The pathogenesis of these tumors is unclear but antecedent trauma and operation have been implicated. Desmoid tumors can present either with pain or be incidentally detected on radiographic studies. We describe the case of a 60-year-old woman with an intrathoracic desmoid detected on a routine screening chest roentgenogram who underwent complete surgical resection of the tumor.
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Introduction
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Desmoid tumors arise from connective tissue sheath of striated muscles. They are locally invasive, have a high recurrence rate after resection, but never metastasize [1]. These tumors are more common in women and are associated with familial polyposis syndromes with the abdomen as the most common site of involvement. Sporadic desmoid tumors involve the abdomen and extra-abdominal sites with equal frequency [1]. Extra-abdominal desmoids are commonly seen in shoulder, chest wall, and thigh [1]. Chest wall involvement comprises 20% of desmoids with a predilection for the anterior chest wall [1]. We report a case of an intrathoracic desmoid, detected incidentally, which mimicked a pulmonary parenchymal lesion.
A 60-year-old woman was referred for evaluation of a computed tomographic scan abnormality. She was a lifelong nonsmoker with no past medical illness, who suffered a motor vehicle accident 9 months prior. Six months later she complained of a new progressively worsening right shoulder pain and underwent a right rotator cuff repair with no relief. She denied all pulmonary or constitutional complaints. She was on no medications, reported serial negative purified protein derivatives, and no specific exposure history. Her physical examination was entirely unremarkable. A computed tomographic scan of the chest (Fig 1) revealed a 3.5-cm apparently parenchymal lesion in the apical segment of right upper lobe, abutting the chest wall. No calcifications, signs of chest wall involvement, mediastinal or hilar adenopathy were detected and the liver and adrenal glands appeared normal. The differential diagnosis at that time included primary lung malignancy, traumatic pulmonary hematoma, and fibroma. She underwent formal thoracotomy, as video-assisted resection was deemed impractical, using at first supraclavicular incision to mobilize an extra-parenchymal soft, rubbery mass firmly adherent to the chest wall in the superior sulcus and then a posterolateral thoracotomy incision to carefully dissect the mass from the surrounding structures. There was no apparent bony invasion. Histology revealed a densely collagenated acellular lesion interspersed with areas of moderate cellularity consisting of fibroblastic and myofibroblastic cells (Fig 2A). In the more cellular areas (Fig 2B) the cells were all of a myofibroblastic type characterized by uniform ovoid vesicular nuclei with punctate eosinophilic nucleoli and lightly basophilic cytoplasm and separated by collagenous fibers. These foci were typical of fibromatosis (desmoid tumor) with complete absence of mitotic figures. There was no infiltration of surrounding bone, nerves, and vessels.
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Fig 1. (A) Right apical mass abutting the chest wall. (B) Computed tomographic scan showing the mass surrounded by lung parenchyma, although operation confirmed its thoracic origin without lung infiltration.
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Fig 2. (A) Area of moderate cellularity showing fibroblasts and myofibroblasts without any mitotic figures. (B) An area of more dense collagen formation with very few cells. (Hematoxylin-eosin, x400.)
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Comment
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Chest wall desmoids usually present with a palpable mass and pain. Nerve involvement can give rise to sensory and motor symptoms in the distribution of the nerve [2]. Most are detectable on chest roentgenogram and may show evidence of bony involvement [2]. Computed tomographic scan usually reveals the size and location; however, magnetic resonance imaging is probably more sensitive in detecting soft tissue infiltration and evidence of local recurrence [3].
The pathogenesis of desmoid tumors is not clear. Trauma has been strongly implicated in the development of abdominal wall desmoids in pregnancy and attributed to local stretching [3]. Chest wall desmoids have been described after mastectomy, silicon breast implants, rib fractures, and thoracotomy [1]. An exuberant inflammatory and reparative response after trauma has been postulated as the potential mechanism. Estrogens also have been associated with the growth of desmoids, especially in association with pregnancy and an apparent therapeutic response to antiestrogen therapy supports this hormonal influence [4].
Definitive diagnosis requires histopathology. Fine needle aspiration may not be sufficient because of the relative hypocellularity of this tumor [3]. Intrathoracic desmoids may present an even more difficult diagnostic problem and, depending on location, neurofibromas, ganglioneuromas, fibrosarcomas, lung cancers, calcifying fibrous pseudotumors, and localized fibrous tumors of the pleura should be considered in the differential diagnosis [5].
Operation remains the first-line treatment for intrathoracic desmoids. Recurrence rates of up to 60% are reported in abdominal desmoid tumor [6]. The recurrence rate in intrathoracic desmoids is not known in view of its rarity. Local recurrence may be avoided by a wide margin of excision beyond the visible tumor. The role of radiotherapy is unclear, but has been used in recurrences and primary extensive tumors believed to be inoperable [7]. Nonsteroidal antiinflammatory drugs in conjunction with ascorbic acid or tamoxifen have been reported to decrease the growth of some desmoids [8].
In our patient, operation confirmed the tumor’s intrathoracic location suggested by computed tomographic scan. Differentiation of desmoid tumors from more common primary lung neoplasms is unlikely on computed tomographic scan alone and particularly difficult when they are located, as in this patient, in the narrow confines of the superior sulcus.
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References
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Abstract
Introduction
