Eur J Cardiothorac Surg 1999;15:723-725
© 1999 Elsevier Science NL
Desmoid fibromatosis of the shoulder and of the upper chest wall following a clavicular fracture
Philippe Icarda,
Jean Philippe Le Rochaisa,
Françoise Galateaub,
Claude Evrarda
a Service de Chirurgie Thoracique, CHRU de Caen, Cote de Nacre, 14033 CaenCedex, France
b Service d'Anatomo-pathologie, CHRU deCaen, France
Received 13 October 1998;
received in revised form 17 February 1999;
accepted 2 March 1999.
Corresponding author. Tel.: +33-1-231063106; fax: +33-1-231064456
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Abstract
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A desmoid tumor of the shoulder girdle infiltrating the upper chest wall and weighing 1500 g was almost completely removed in an 18-year-old man, 27 months after a bifocal fracture of the clavicule. Thirteen years later, the patient was free of recurrence. The interval time between trauma and diagnosis, as the particular characteristics of aggressive fibromatosis, strongly support a major causal role of the clavicular fracture in the occurrence of this tumor.
Key Words: Desmoid tumor • Aggressive fibromatosis • Keloid
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1. Introduction
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Desmoid tumor is an aggressive deep fibromatosis that may occur in abdominal, intra-abdominal, and extra-abdominal areas [1–4]. Local recurrence after resection is frequent, but it almost never metastases [1–4]. Although causative relationship with previous trauma is frequently proposed [1–3], occurrence after bone fracture remains exceptional [5]. We present herein a case of desmoid tumor of the shoulder infiltrating the upper chest wall, diagnosed 2 years after a clavicular bone fracture.
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2. Patient
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A 15-year-old boy was a victim of a motorcycle accident in October 1980. He suffered from a displaced bifocal fracture of the left clavicule, which was immobilized for 1 month with a bandage, leading to complete healing with a residual callus. Two years later, the patient was complaining of pain in his shoulder. A mass of the subclavicular area, about 8 cm in diameter, was biopsied in another hospital. Histological examination revealed a desmoid tumor, with fascicles of benign-appearing spindle cells. Five months later, in February 1983, the patient was referred to our center with a very large firm mass, not adherent to the skin, but fixed and infiltrating to the underlying tissues and muscles, extending from the supraclavicular area to the subclavicular, upper chest wall and axillary areas, and compressing vessels. The tumor was firmly attached to the clavicule. A CT scan tried to determine the exact limits of the lesion (Fig. 1) and an angiography vizualized patent subclavian and axillary vessels, that were severely displaced and compressed (Fig. 2)
. The tumor was dissected through a large anterior and transverse cervical and axillary skin incision. Control of vessels need to resect the entire infiltrated clavicle. Near complete resection of the tumor was performed in two large enbloc masses, with sacrifice of infiltrated pectoralis muscles, as parts of the deltoid, of the first and second ribs, and of the scapula. The two en bloc tumors measured, respectively 10.5x5x5 cm and 15x16x7 cm, both weighing 1500 g. Free margins were achieved all around, except in contact with the scapula. Larger resection was rejected because it would have led to a forearm amputation. Post-operative course was uneventful. No complementary treatment was given. After 13 years of follow-up with regular CT scans, the patient was free of recurrence, suffering principally of esthetic sequelaes, due to an anterior thoracic deformation. Although the patient is limited in abduction, he had a normal strength and sensibility of his upper limb.
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Fig. 2. Angiography, showing the displacement of the axillary vessels by the tumor. CH, chest wall; C, clavicula; S, scapula; AA, axillary artery; T, tumor.
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3. Discussion
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Desmoid tumor or aggressive fibromatosis is extremely rare, with less than five cases per million inhabitants [3]. In extra-abdominal areas, the shoulder girdle is the commonest location, frequently seen in adolescent and young adults, as in our patient [1–3]. Pain, and symptoms of nerve or vascular compression occur when the lesion becomes voluminous. As in our case, the lesion is usually poorly circumscribed, infiltrating adjacent bone and muscles, leading to great difficulties in determining the exact boundaries, even, during operation [1,2]. Histological analysis must differentiate aggressive fibromatosis from nodular fasciitis (generally less than 5 cm in size), and from low-grade fibrosarcoma [1,2]. Nodular fasciitis is often also associated with trauma but is characterized by a ‘feathery pattern’ with hemosiderin. Low-grade fibrosarcoma is usually well encapsulated with distinct abnormal mitoses. Several etiologic factors have been proposed to explain fibromatosis: trauma will activate occurrence, genetic disorder will predispose to occurrence, and estrogen will influence tumor growth [1–3]. History of previous trauma is frequently reported. In 89 desmoid intra-abdominal tumors, Reitamo et al. [3] reported a 30% rate of occurrence in the 4 years after a previous operation. In 30 cases concerning the shoulder girdle, a history of trauma was reported in 63% of the cases, but only nine cases (30%) were considered to show a direct relationship between tumor and trauma [1]. As in our patient, the interval time between trauma and diagnosis of fibromatosis is usually 2 years [1–3,5], a delay according well with the tumor growing rate of 7.4±1.7 cm/year, calculated in male patients by Reitamo et al. [3]. Although it remains almost impossible to prove with certainty an indisputable relationship between trauma and fibromatosis, the high incidence of preceding injury in desmoid fibromatosis is greater than that of soft tissue tumors in general [1–3]. In extra-abdominal tumors, a high linkage with surgical trauma has not been reported, with few cases occurring after thoracotomy [1,2]. Fibromatosis occurring after fracture is still more rare, and only reported in a patient by Urist et al. [5], occurring similarly to our case, 2 years after a fracture of the forearm. Trauma may be supposed as a causal agent of activating growth repair factors, revealing a preexisting defect [1–3]. Because intra-abdominal desmoid tumors are extremely common among patients with Gardner‘s syndrome, the genes regulating these two disorders should be closely related, but different, as less than 2% of patients with desmoid tumor have polyposis coli [3]. Because desmoid tumors have been produced in rats with estrogen, and would have a faster rate of growth in fertile women, an hormonal influence has been suspected [3]. However, until today, few responses have been reported under hormonal therapy [2]. Prognosis factors lack to predict the clinical course of patients. Resection is the best option but recurrences are numerous, especially when it is incomplete [1–4], usually within 2 years of operation, with a rate ranging from 25 to 68%. Adequate margin of resection cannot be always easily obtained, due to the need of preserving vital or important structures, and due to the lack of encapsulation makes often difficult for the surgeon to distinguish clearly between tumor and scar tissue [1,2]. Furthermore, spontaneous regression or no recurrence following incomplete resection, as in our case, may occur, with an incidence rate of 20% in one study [4]. Complementary radiotherapy was reported as effective in preventing recurrence in 10 patients with narrow margins [6]. Because in axillary area, radiotherapy may lead to lymphoedema, we did not administer radiotherapy in your young patient, who remained free of recurrence 13 years later. When the tumor recurs and/or cannot be excised, a forearm amputation must be considered or high doses of radiotherapy and possibly chemotherapy, although outcome under this condition is not well known. Finally, the desmoid tumor is an aggressive fibromatosis that remains a very interesting but poorly understood neoplasm.
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References
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- Enzinger F.M., Shiraki M. Musculo-aponeurotic fibromatosis of the shoulder girdle (extra-abdominal desmoid). Analysis of thirty cases followed up for ten or more years. Cancer 1967;20:1131-1140.
- Enzinger F.M., Weiss S.W. Soft tissue tumors. St Louis: Mosby 1983:45-102.
- Reitamo J.J., Scheinin T.M., Hayry P. The desmoid syndrome: new aspects in the cause, pathogenesis and treatment of the desmoid tumor. Am J Surg 1986;151:230-237.[Medline]
- Posner M.C., Shiu M.H., Newsome J.L., Hajdu S.I., Gaynor J.J., Brennan M.F. The desmoid tumor. Not a benign disease. Arch Surg 1989;124:191-196.
- Urist M.R. Trauma and neoplasm: report of a case of desmoid tumor following simple fracture of radius and ulna. Am J Surg 1957;93:682-693.[Medline]
- Karakousis C.P., Mayordomo J., Zografos G.C., Driscoll D.L. Cancer 1993;72:1637-1641.[Medline]
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Abstract
1. Introduction
