Ann Thorac Surg 2006;82:342-345
© 2006 The Society of Thoracic Surgeons
Case report
Recurrent Localized Fibrous Tumor of the Pleura
Balakrishnan Mahesh, MS, FRCS
a
,
*
,
Colin Clelland, FRCPath
b
,
Chandana Ratnatunga, FRCS(CTh)
a
a Division of Thoracic Surgery, Oxford, United Kingdom
b Division of Pathology, John Radcliffe Hospital, Oxford, United Kingdom
Accepted for publication September 20, 2005.
* Address correspondence to Dr Mahesh, Transplant Immunology Heart Science Center, Harefield Hospital, Harefield, UB96JH United Kingdom (Email: b.mahesh{at}imperial.ac.uk).
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Abstract
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Localized fibrous tumors of the pleura are rare. They are often asymptomatic and may have symptoms based on size, bronchial invasion, or hormone production, or a combination of these. Complete resection offers the best chance of cure. However, recurrence is reported in a significant number of patients and can often be treated by repeated resection, albeit with increasing difficulty. We present a case in which delayed recurrence occurred after excision of such a tumor. This required a chest-wall resection and reconstruction after which a second recurrence occurred. Further thoracotomy including a latissimus dorsi free flap procedure was needed for a third-time recurrence.
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Introduction
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Localized fibrous tumors of the pleura (LFTP) are rare [1–5]. They originate from primitive mesenchymal cells in submesothelial tissue [1, 4]. Benign and malignant forms have been defined by histological criteria [4]. These tumors may recur despite complete excision and benign histology. Conversely, those classified as malignant, may be cured by complete resection [5]. We present a case of recurrent LFTP, in which the pathology evolved to increasingly malignant forms with each subsequent recurrence.
A 49-year-old woman presented in January 2003 with recurrent right intrathoracic tumor. She had a history of recurrent fasting hypoglycemic attacks with documented blood-sugar levels of 40 mg/dL.
Previously she had undergone a right thoracotomy in 1991 for LFTP. The lesion had recurred in 2001, requiring re-resection. During the operation three large masses were found and removed off the diaphragm. She made an unremarkable recovery.
Based on computerized tomographic scans showing a recurrent tumor, she underwent redo-thoracotomy (third-time) in March 2003. An 8-cm tumor, adherent to the chest wall and diaphragm, was found and removed. Parts of the sixth, seventh, eighth, and ninth ribs, adherent to the mass, were removed. A portion of the diaphragm from which the tumor arose was excised and repaired with polypropylene mesh. The chest-wall defect was repaired with polymethylmethacrylate cement, sandwiched between two layers of polypropylene mesh (Fig 1a).
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Fig 1. Computed tomographic scans showing (a) chest-wall reconstruction with 2 sandwiches of polypropylene mesh and cement (arrowheads), and (b) recurrent tumor adjacent to posterior ribs (arrowhead). Both scans were taken 10 months after third-time resection. Absence of adhesions between the tumor and the polypropylene mesh allowed this to be reflected anteriorly at fourth-time resection. (c) Unfortunately, infection necessitated removal of the mesh and replacement with latissimus-dorsi free flap (arrowhead).
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Histology revealed the lesion to be a malignant transformation of LFTP with abnormal mitoses ranging from 1 to 5 per 10 high-power fields and nuclear pleomorphism (Figs 2a,
2b). The specimen was immunohistochemically positive for S100, CD34, CD99, BCL2, p53, and desmins. The ribs and diaphragm were tumor free.
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Fig 2. Representative high-power sections showing (a) abnormal mitoses (arrowhead) and (b) nuclear pleomorphism (arrowheads) in the tumor removed at third-time resection. Sections from the tumor removed at fourth-time resection (c) revealed increasing nuclear pleomorphism and myxoid stroma (arrowheads), suggestive of increasing malignant change.
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She presented 10 months later with recurrent tumor adjacent to the posterior ribs (Fig 1b). She underwent a redo-thoracotomy (4th time) in January 2004. Access was obtained by reflecting the previously implanted polypropylene mesh anteriorly; the encapsulated tumor was mobilized and resected. Recovery was complicated by osteomyelitis of the ribs and infection of the mesh, all of which were removed and replaced with a Latissimus Dorsi muscle free-flap (Fig 1c), which was anastomosed to the thoracodorsal vessels. She then underwent a slow recovery. Histopathology revealed a spindle-cell tumor with myxoid stroma, increased nuclear pleomorphism, mitoses, and prominent blood vessels, all suggestive of malignant change (Fig 2c).
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Comment
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There are two types of primary pleural tumors: (1) diffuse and (2) solitary [1, 6]. Diffuse variety is the malignant mesothelioma, which is related to asbestos exposure and has dismal prognosis. Localized fibrous tumors of the pleura are rare and asymptomatic [2, 5]. No sex predominance or relationship to asbestos exposure has been reported [1, 2, 4, 6]. Symptoms depend on size and erosion into adjacent structures, and include cough, hemoptysis, dyspnea, fever, weight loss, and anorexia [1, 2]. Symptoms are more common in malignant varieties [4]. Recurrent hypoglycemia, as in our case, is seen in 2% to 4% of cases, due to production of insulin-like growth-factor-2 by the tumor [1, 6], and is more common in women [4]. Others (
20%) may develop hypertrophic pulmonary osteoarthropathy [1, 2, 6].
Benign variants constitute more than 80% of LFTP [6]. England and co-workers [4] described three histopathologic characteristics associated with malignancy: (1) hypercellularity, crowding, and overlapping of nuclei, (2) nuclear pleomorphism and nucleolar prominence, or (3) high mitotic activity 
4 mitoses/10 high-power fields (even if present focally), or a combination of these. Pleomorphic giant cells and abnormal mitotic activity were also indicative of malignancy. They found that high mitotic rates had good correlation with myxomatous changes, hemorrhage, and necrosis.
A combination of clubbing, hypertrophic pulmonary osteoarthropathy, and hypoglycemia, in conjunction with a large intrathoracic mass should raise the suspicion of LFTP [6]. Radiologic investigations and even computerized tomographic-guided biopsies may not be useful in differentiating LFTP from more commonly encountered primary lung malignancies, especially in the presence of history of smoking [5]. These investigations are also frequently unhelpful in differentiating between benign and malignant pleural tumors [3]. Magnetic resonance imaging may reveal the fibrous character of the lesion (eg, vascular channels seem to be serpiginous signal voids) [7]. Fine-needle aspiration cytology is unhelpful in achieving a diagnosis [2, 3, 6], but Trucut biopsy may be useful [8]. The tumors invariably grow in size, and many produce symptoms. Therefore an operation is required for both diagnosis and treatment.
Optimal treatment entails complete excision, including lung resection if the tumor arises from visceral pleura, and chest-wall and pericardial resection when required, with microscopically free resection margins confirmed intraoperatively [2–6]. Broad-based tumors arising from parietal pleura may require extrapleural resection [6]. Chest-wall invasion does not equate to malignant tumor, and chest-wall resection should be performed when deemed necessary.
There have been several series reporting the surgical management of LFTP with good results [1–6], but recurrences occur in both benign and malignant disease. Recurrence has been reported after complete excision of benign tumors, sometimes at lengthy intervals after surgery. The probability of recurrence with malignant disease is greater, but complete excision offers the best possibility of cure in malignant forms [3, 5, 6]. Incomplete surgical excision is the most important predictor of recurrence and of survival [5]. Other factors include histology, size, and base of the lesion [2]. Local recurrence has been reported even as late as 17 years after an operation. Re-resection is indicated in these cases, and when possible this does not adversely affect survival in patients with locally-recurrent benign tumors [5]. More aggressive histologic features suggestive of malignant change, including fibrosarcomatous changes [3], may appear with each recurrence [5]. Radiotherapy and chemotherapy have been undertaken for locally recurrent malignant tumors with possibly good long-term results [2–4].
Our case recurred 10, 12, and 13 years after complete resection of benign LFTP. Histology revealed malignant transformation of benign LFTP during the third and fourth re-resections. The patient made an excellent recovery after second thoracotomy, only to have recurrence 2 years later, with the tumor involving the chest-wall and diaphragm, and requiring resection and reconstruction. Histology confirmed its malignant nature, and the tumor margins were found to be free from disease. In view of rapidity of recurrence in this instance, postoperative radiotherapy was considered, but it was not instituted because of the paucity of evidence for it in the literature. Further recurrence was observed, requiring a fourth thoracotomy and free-flap procedure for infection and osteomyelitis. Radiotherapy is currently being withheld because the patient required a free-flap procedure to treat osteomyelitis, as well as lack of concrete proof in the literature favoring radiotherapy. Instead the patient is being closely followed-up, which is the appropriate line of management for these patients.
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References
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Abstract
Introduction
