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Desmoplastic fibroma of the rib
Case Report Desmoplastic Fibroma Of The Rib Kwang Ho Kim, MD 1, Jae Yul Han,
MD
2, Hyung Kook Kim,
MD
2, Woon Sup Han,
MD
3
IDepartment of Thoracic and Cardiovascular Surgery, Inha University Hospital, Inchon, Republic of Korea; 2Department of Thoracic and Cardiovascular Surgery, Ehwa Womans University, Mokdong Hospital, Seoul, Republic of Korea; 3Department of Pathology, Ewha Womans University Mokdong Hospital, Seoul, Republic of Korea Desmoplastic fibroma of the bone is a rare benign tumour which resembles the desmoid tumour of the abdominal wall. It is rarely found in the rib although it is found in the metaphysis of the long bones and facial bones. We report a 22-year-old female patient with desmoplastic llbroma in the left 10th rib who was treated with wide surgical resection of the affected rib with good clinical results. She has been followed for one and one-half years after the resection with no evidence of recurrence. (Asia Paciilc Heart Journal 19!98;7(2):12%130)
Introduction Desmoplastic fibroma of the bone, which is histologically similar in appearance to the desmoid tumour of the abdominal wall, is a rare benign tumour. It is not usually found in the rib, and usually develops in the metaphysis of the long bones, short flat bones, and facial bones.132We present an unusual case in which a desmoplastic fibroma developed in a rib.
Case Report A 22-year-old female patient was admitted to the Ewha Womans University Mokdong Hospital, Seoul, Korea, with a complaint of dull pain in the left lower chest wall for 1 year. On physical examination an immobile, non-tender mass, 3 cm in diameter, was palpable anterolaterally in the left 10th rib. X-ray examination of the chest with rib views showed a sharply marginated, osteolytic lesion in the anterior segment of the left 10th rib without definite sclerotic rim or cortical destruction (Fig. 1).
Fig. 1. Sharply marginated and osteolytic lesion in the anterior segment of the left 10th rib. periphery of the lesion. There were no mitotic figures and minimal nuclear atypia and prominent nucleoli (Figs. 2 and 3). Postoperative recovery was uneventful. There has been no recurrence after one and one-half years. Discussion Desmoplastic fibroma of the bone is a rare tumour, although it does develop in the metaphysis of the long bones (femur, tibia) or flat bones (pelvis, facial bones, spine).QJ However, a desmoplastic fibroma originating in the chest wall is rare. A thorough search of the literature revealed only 2 cases of rib involvement 495 and 2 cases of sternal involvement.677 Histologically, desmoplastic fibroma has an abundant extacellular matrix of collagen fibres with uniform spindle-shaped fibroblasts which contain an oval nucleolus. Atypical nuclei and mitoses are not observed.2
Bone scan showed increased uptake in the same segment of the rib. Wide surgical resection of the tumour was carried out under general anaesthesia. The irregular tumour was 3x3 cm in size and adherent to the parietal pleura. It had a thin cortex, and histologically the tumour was composed of spindle cells having features of fibroblasts. The proliferating spindle cells were separated by abundant collagen fibres. There were also scattered thin-walled vessels. No irregularly shaped trabeculae of woven bone in a background of fibrous tissue were noted. Entrapped remnants of bone were noted in the
129
Kim, Han, Kim, Han Desmoplasticfibroma of rib
Fig. 2. Histology of tumour showing diffuse proliferation of fibroblasts, abundant collagen fibres, and a medium sized vessel.(Haematoxylin-eosin; x200 without reduction).
Fig. 3. Remnant of bone is noted in the periphery of the
Desmoplastic fibroma resembles the desmoid tumor of the fascia of the abdominal wall in showing small spindleshaped fibroblasts sparsely scattered throughout the lesion, interposed between thick intercellular collagen fibres.2 It can be difficult to distinguish from a low-grade fibrosarcoma and fibroblastic osteosarcoma which show focal nuclear pleomorphism, more than 5 mitoses per 10 high-power fields and/or atypical mit0ses.s The symptoms of a desmoplastic fibroma are often vague, and of long duration, with pain and swelling, or tenderness due to a pathological fracture.2 The present patient had dull pain in the left chest for 1 year. Radiologically this tumour usually shows a localised cystic tumour in the middle of a metaphysis with a radiolucent central area and trabecular structure. The cortex layer around the tumour is usually thin and of irregular structure. The radiological appearances of desmoplastic fibroma are often confused with other benign or malignant bone lesions such as giant cell tumour, fibrous dysplasia, chondromyxoid fibroma, non-ossifying fibroma and simple bone cyst.8
Although desmoplastic fibroma is benign (in that it is non-metastasising), it is locally aggressive. The correct therapy is therefore wide local excision of the tumour. In this case, wide excision of the rib (including the tumour) was performed without local recurrence for one and a half years.
tumour, which is characterised by uniform spindle proliferation. (Haematoxylin-eosin; xl00 without reduction).
References 1. 2. 3. 4. 5. 6. 7. 8.
130
Rabhan WN, Rosai J. Desmoplastic fibroma. J Bone Joint Surg 1968;50A:487-502. Gebhardt MC, Cambell C J, Schiller AL, Mankin HJ. Desmoplastic fibroma of bone. J Bone Joint Surg 1985;67A:73247. Shinomiya K, Furuya K, Mutoh N. Desmoplastic fibroma in the thoracic spine. J Spinal Disord 1991;4:229-33. Schajowicz F. Tumors and tumor like lesions of bone and joints. New York: Springer, 1981;335-9. Butters M, Hamann H, Mohr W. Desmoplastic fibroma of the rib. Thorac Cardiovasc Surgeon 1985;33:317-8. Waddell WR, Gemer RE. Indomethacin and ascorbate inhibit desmoit tumours. J Surg Oncology 1980;15:85-90. Obaro ROI. Desmoplastic fibroma of the sternum. Clin Radio1 1992;46:359-60. Crim JR, Gold RH, Mirra JM, Eckardt JJ, Bassett LW. Desmoplastic fibroma of bone: radiological analysis. Radiology 1989:172:827-32.
MD
2, Hyung Kook Kim,
MD
2, Woon Sup Han,
MD
3
IDepartment of Thoracic and Cardiovascular Surgery, Inha University Hospital, Inchon, Republic of Korea; 2Department of Thoracic and Cardiovascular Surgery, Ehwa Womans University, Mokdong Hospital, Seoul, Republic of Korea; 3Department of Pathology, Ewha Womans University Mokdong Hospital, Seoul, Republic of Korea Desmoplastic fibroma of the bone is a rare benign tumour which resembles the desmoid tumour of the abdominal wall. It is rarely found in the rib although it is found in the metaphysis of the long bones and facial bones. We report a 22-year-old female patient with desmoplastic llbroma in the left 10th rib who was treated with wide surgical resection of the affected rib with good clinical results. She has been followed for one and one-half years after the resection with no evidence of recurrence. (Asia Paciilc Heart Journal 19!98;7(2):12%130)
Introduction Desmoplastic fibroma of the bone, which is histologically similar in appearance to the desmoid tumour of the abdominal wall, is a rare benign tumour. It is not usually found in the rib, and usually develops in the metaphysis of the long bones, short flat bones, and facial bones.132We present an unusual case in which a desmoplastic fibroma developed in a rib.
Case Report A 22-year-old female patient was admitted to the Ewha Womans University Mokdong Hospital, Seoul, Korea, with a complaint of dull pain in the left lower chest wall for 1 year. On physical examination an immobile, non-tender mass, 3 cm in diameter, was palpable anterolaterally in the left 10th rib. X-ray examination of the chest with rib views showed a sharply marginated, osteolytic lesion in the anterior segment of the left 10th rib without definite sclerotic rim or cortical destruction (Fig. 1).
Fig. 1. Sharply marginated and osteolytic lesion in the anterior segment of the left 10th rib. periphery of the lesion. There were no mitotic figures and minimal nuclear atypia and prominent nucleoli (Figs. 2 and 3). Postoperative recovery was uneventful. There has been no recurrence after one and one-half years. Discussion Desmoplastic fibroma of the bone is a rare tumour, although it does develop in the metaphysis of the long bones (femur, tibia) or flat bones (pelvis, facial bones, spine).QJ However, a desmoplastic fibroma originating in the chest wall is rare. A thorough search of the literature revealed only 2 cases of rib involvement 495 and 2 cases of sternal involvement.677 Histologically, desmoplastic fibroma has an abundant extacellular matrix of collagen fibres with uniform spindle-shaped fibroblasts which contain an oval nucleolus. Atypical nuclei and mitoses are not observed.2
Bone scan showed increased uptake in the same segment of the rib. Wide surgical resection of the tumour was carried out under general anaesthesia. The irregular tumour was 3x3 cm in size and adherent to the parietal pleura. It had a thin cortex, and histologically the tumour was composed of spindle cells having features of fibroblasts. The proliferating spindle cells were separated by abundant collagen fibres. There were also scattered thin-walled vessels. No irregularly shaped trabeculae of woven bone in a background of fibrous tissue were noted. Entrapped remnants of bone were noted in the
129
Kim, Han, Kim, Han Desmoplasticfibroma of rib
Fig. 2. Histology of tumour showing diffuse proliferation of fibroblasts, abundant collagen fibres, and a medium sized vessel.(Haematoxylin-eosin; x200 without reduction).
Fig. 3. Remnant of bone is noted in the periphery of the
Desmoplastic fibroma resembles the desmoid tumor of the fascia of the abdominal wall in showing small spindleshaped fibroblasts sparsely scattered throughout the lesion, interposed between thick intercellular collagen fibres.2 It can be difficult to distinguish from a low-grade fibrosarcoma and fibroblastic osteosarcoma which show focal nuclear pleomorphism, more than 5 mitoses per 10 high-power fields and/or atypical mit0ses.s The symptoms of a desmoplastic fibroma are often vague, and of long duration, with pain and swelling, or tenderness due to a pathological fracture.2 The present patient had dull pain in the left chest for 1 year. Radiologically this tumour usually shows a localised cystic tumour in the middle of a metaphysis with a radiolucent central area and trabecular structure. The cortex layer around the tumour is usually thin and of irregular structure. The radiological appearances of desmoplastic fibroma are often confused with other benign or malignant bone lesions such as giant cell tumour, fibrous dysplasia, chondromyxoid fibroma, non-ossifying fibroma and simple bone cyst.8
Although desmoplastic fibroma is benign (in that it is non-metastasising), it is locally aggressive. The correct therapy is therefore wide local excision of the tumour. In this case, wide excision of the rib (including the tumour) was performed without local recurrence for one and a half years.
tumour, which is characterised by uniform spindle proliferation. (Haematoxylin-eosin; xl00 without reduction).
References 1. 2. 3. 4. 5. 6. 7. 8.
130
Rabhan WN, Rosai J. Desmoplastic fibroma. J Bone Joint Surg 1968;50A:487-502. Gebhardt MC, Cambell C J, Schiller AL, Mankin HJ. Desmoplastic fibroma of bone. J Bone Joint Surg 1985;67A:73247. Shinomiya K, Furuya K, Mutoh N. Desmoplastic fibroma in the thoracic spine. J Spinal Disord 1991;4:229-33. Schajowicz F. Tumors and tumor like lesions of bone and joints. New York: Springer, 1981;335-9. Butters M, Hamann H, Mohr W. Desmoplastic fibroma of the rib. Thorac Cardiovasc Surgeon 1985;33:317-8. Waddell WR, Gemer RE. Indomethacin and ascorbate inhibit desmoit tumours. J Surg Oncology 1980;15:85-90. Obaro ROI. Desmoplastic fibroma of the sternum. Clin Radio1 1992;46:359-60. Crim JR, Gold RH, Mirra JM, Eckardt JJ, Bassett LW. Desmoplastic fibroma of bone: radiological analysis. Radiology 1989:172:827-32.