- Email: [email protected]
Atypical palmar fibromatosis with giant fibrous nodule: A case report
Atypical Palmar Fibromatosis with Giant Fibrous Nodule: A Case Report Keiichi Muramatsu, MD, Koichiro Ihara, MD, Shinya Kawai, MD, Yamaguchi, Japan
We present an unusual case of palmar fibromatosis. A 62-year-old man had a giant fibrous nodule in the deep fascia of the right palm without any pretendinous cord or flexion deformity at the metacarpophalangeal joint. Primarily soft-tissue tumor and palmar fibromatosis were considered as differential diagnosis of nodular mass but finally the latter was confirmed histologically after resection of the giant nodule. (J Hand Surg 2003;28A:525-527. Copyright © 2003 by the American Society for Surgery of the Hand.) Key words: Palmar fibromatosis, giant fibrous nodule, extra-abdominal desmoid, differential diagnosis.
The palmar nodule usually is seen in the early stage of Dupuytren’s disease and is the key for an early diagnosis.1 As the disease progresses the palmar nodule gradually regresses and this is followed by the development of pretendinous and digital cords.2 We report a case of fibromatosis with a giant palmer nodule that did not represent the typical case of Dupytren’s disease. Soft-tissue tumor such as extraabdominal desmoid (deep fibromatosis), epitheloid sarcoma, fibrosarcoma and giant cell tumor, and pigmented villonodular synovitis were considered as differential diagnosis. The definite diagnosis was confirmed histologically as palmar fibromatosis.
From the Department of Orthopedic Surgery, Yamaguchi University School of Medicine, Yamaguchi, Japan. Received for publication May 31, 2002; accepted in revised form December 16, 2002. No benefits in any form have been received or will be received from a commercial party related directly or indirectly to the subject of this article. Reprint requests: Keiichi Muramatsu, MD, Department of Orthopedic Surgery, Yamaguchi University School of Medicine, 1-1-1 MinamiKogushi, Ube, Yamaguchi 755-8505, Japan Copyright © 2003 by the American Society for Surgery of the Hand 0363-5023/03/28A03-0026$30.00/0 doi:10.1053/jhsu.2003.50075
Case Report A 62-year-old Japanese man developed a painless, firm, soft-tissue mass at the right distal palmar crease in line with the third web space 6 years before visiting our clinic. The patient came to our clinic because of the onset of pain and acute enlargement of the palmar mass over the past 3 months. He had no mass in the left hand and had no history of diabetes mellitus, epilepsy, alcoholism, or trauma, and no ectopic lesions of fibromatosis were detected either. He had no family history of palmar fibromatosis or neoplasm. On examination there was a palpable lump in the palm without any cord-like thickening. This lump was painful on palpation and its size was 6 ⫻ 6 cm (Fig. 1). There was no flexion contracture of the metacarpophalangeal or proximal interphalangeal joints. Sensation in the middle and ring finger was disturbed mildly. Before surgery soft-tissue sarcoma and palmer fibromatosis were considered rather than benign soft-tissue tumor because the palmer mass acutely re-enlarged in the past 3 months. At surgery intraoperative histopathology was first performed through a limited incision on the top of the mass to confirm the definite diagnosis. Pathologic examination revealed the cellular and vascular nature The Journal of Hand Surgery
525
526
The Journal of Hand Surgery / Vol. 28A No. 3 May 2003
Figure 2. Histopathology showing the cellular and vascular nature of the nodule rich with proliferation of collagen fibers. The fibroblasts were less numerous and aligned parallel to one another. The nodule contained markedly increased amounts of dense collagen fibers. Microscopic findings revealed no malignant findings.
Figure 1. A palpable lump (6 ⫻ 6 cm) in the palm. There was no cord-like thickening and no flexion contracture of either the metacarpophalangeal or proximal interphalangeal joints.
of the nodule, rich with proliferation of collagen fibers, and it was diagnosed as palmar fibromatosis grade II according to Luck’s3 classification (Fig. 2). Through a wide Bruner4 zigzag incision the giant fibrous nodule was dissected from the surrounding tissue and successfully excised. The nodule was found to exist beneath the neurovascular bundle and flexor tendons and extended from the base of the middle finger to the distal end of the transverse carpal ligament (Fig. 3). The tumor arose from the deepest longitudinal fibers that passed deep to either side of the flexor tendons. No pretendinous or digital cords were found during surgery.
Discussion Fibromatoses are a broad group of benign fibrous tissue proliferations of similar microscopic appearance that are intermediate in their biologic behavior between benign fibrous lesions and fibrosarcoma. The fibromatoses can be divided into 2 major groups. Superficial fibromatoses arise from the fascia or apo-
Figure 3. The giant palmar nodule (single arrow) revealed a round surface and was found to exist beneath the flexor tendons (double arrows) and neurovascular bundle (triple arrows) and extended from the base of the middle finger to the distal end of the transverse carpal ligament. The nodule was firm and scar-like on palpation and revealed a uniformly gray-white surface. The palmar aponeurosis was damaged partially by the giant nodule but no pretendinous or digital cords were found during surgery.
Muramatsu, Ihara, and Kawai / Giant Fibrous Nodule
neurosis and only rarely involve deeper structure. Deep fibromatosis (extra-abdominal desmoid) tend to be more aggressive in their behavior than the superficial fibromatoses and principally involve deeper structures, particularly the musculature of the trunk and the extremities.5 In this case, the tumor arose from the longitudinal fibers that passed deep to either side of the flexor tendons, we diagnosed this as palmar fibromatosis. Palmar fibromatosis belongs to the superficial fibromatoses group and its clinical course and progression are not uniform. McFarlane et al. divided the disease progression into 3 stages.6 – 8 In the classic scenario of typical disease a nodule develops in the palm and digits (stage I), followed by the formation of a pretendinous and digital cord that gradually contracts (stage II), leading to progressive digital flexion deformity (stage III). Although the origin of the palmar nodule is uncertain Rayan2 presented in his review that it seems to originate in the superficial components of the palmar fascia and its vertical fibers extend into the dermis. Apparently this patient’s presentation and progression was not compatible with that of typical Dupuytren’s disease. The giant palmar nodule was found to exist beneath the neurovascular bundle and between the flexor tendon and metacarpal bones. McGrouther divided the components of the palmer fascia into 3 layers.9 Layer 1 is the most superficial fibers inserting into the skin, layer 2 is the spiral fibers passing on either side of the flexor tendon, and layer 3 is the deepest longitudinal fibers passing deeply on either side of the flexor tendons and on either side of the metacarpal bones. In this case the
527
giant palmar nodule seemed to originate from McGrouther’s layer 3. The palmar nodule often regresses gradually giving way to development of a cord (stage I to II). The surgical findings revealed that the nodule grew like a soft-tissue tumor without development of any pretendinous cord or flexion deformity of the digits. Palmar fibrosis is uncommon in non-Caucasian races.10 In this case the patient had no risk factors such as diabetes mellitus, epilepsy, alcoholism, or trauma,11 and no ectopic fibromatosis was noted.
References 1. Rayan GM. Clinical presentation and types of Dupuytren’s disease. Hand Clin 1999;15:87–96. 2. Rayan GM. Palmar fascial complex anatomy and pathology in Dupuytren’s disease. Hand Clin 1999;15:73– 86. 3. Luck JV. Dupytren’s contracture. A new concept of the pathogenesis correlated with surgical management. J Bone Joint Surg 1959;41A:635– 664. 4. Bruner JM. The zig-zag volar-digital incision for flexortendon surgery. Plast Reconstr Surg 1967;40:571–574. 5. Enzinger FM. Fibromatosis. In: Enzinger FM, Weiss SW, eds. Soft tissue tumors. St. Louis: Mosby; 1994:201–230. 6. Caughell KA, McFarlane RM, McGrouther DA, Martin AH. Developmental anatomy of the palmar aponeurosis and its relationship to the palmaris longus tendon. J Hand Surg 1988;13A:485– 493. 7. McFarlane RM. Progress in Dupuytren’s disease [Editorial]. J Hand Surg 1991;16B:237–239. 8. McFarlane RM. The current status of Dupuytren’s disease. J Hand Surg 1983;8:703–708. 9. McGrouther DA. The microanatomy of Dupuytren’s contracture. Hand 1982;14:215–236. 10. Burge P. Genetics of Dupuytren’s disease. Hand Clin 1999; 15:63–71. 11. Ross DC. Epidemiology of Dupuytren’s disease. Hand Clin 1999;15:53– 62.
We present an unusual case of palmar fibromatosis. A 62-year-old man had a giant fibrous nodule in the deep fascia of the right palm without any pretendinous cord or flexion deformity at the metacarpophalangeal joint. Primarily soft-tissue tumor and palmar fibromatosis were considered as differential diagnosis of nodular mass but finally the latter was confirmed histologically after resection of the giant nodule. (J Hand Surg 2003;28A:525-527. Copyright © 2003 by the American Society for Surgery of the Hand.) Key words: Palmar fibromatosis, giant fibrous nodule, extra-abdominal desmoid, differential diagnosis.
The palmar nodule usually is seen in the early stage of Dupuytren’s disease and is the key for an early diagnosis.1 As the disease progresses the palmar nodule gradually regresses and this is followed by the development of pretendinous and digital cords.2 We report a case of fibromatosis with a giant palmer nodule that did not represent the typical case of Dupytren’s disease. Soft-tissue tumor such as extraabdominal desmoid (deep fibromatosis), epitheloid sarcoma, fibrosarcoma and giant cell tumor, and pigmented villonodular synovitis were considered as differential diagnosis. The definite diagnosis was confirmed histologically as palmar fibromatosis.
From the Department of Orthopedic Surgery, Yamaguchi University School of Medicine, Yamaguchi, Japan. Received for publication May 31, 2002; accepted in revised form December 16, 2002. No benefits in any form have been received or will be received from a commercial party related directly or indirectly to the subject of this article. Reprint requests: Keiichi Muramatsu, MD, Department of Orthopedic Surgery, Yamaguchi University School of Medicine, 1-1-1 MinamiKogushi, Ube, Yamaguchi 755-8505, Japan Copyright © 2003 by the American Society for Surgery of the Hand 0363-5023/03/28A03-0026$30.00/0 doi:10.1053/jhsu.2003.50075
Case Report A 62-year-old Japanese man developed a painless, firm, soft-tissue mass at the right distal palmar crease in line with the third web space 6 years before visiting our clinic. The patient came to our clinic because of the onset of pain and acute enlargement of the palmar mass over the past 3 months. He had no mass in the left hand and had no history of diabetes mellitus, epilepsy, alcoholism, or trauma, and no ectopic lesions of fibromatosis were detected either. He had no family history of palmar fibromatosis or neoplasm. On examination there was a palpable lump in the palm without any cord-like thickening. This lump was painful on palpation and its size was 6 ⫻ 6 cm (Fig. 1). There was no flexion contracture of the metacarpophalangeal or proximal interphalangeal joints. Sensation in the middle and ring finger was disturbed mildly. Before surgery soft-tissue sarcoma and palmer fibromatosis were considered rather than benign soft-tissue tumor because the palmer mass acutely re-enlarged in the past 3 months. At surgery intraoperative histopathology was first performed through a limited incision on the top of the mass to confirm the definite diagnosis. Pathologic examination revealed the cellular and vascular nature The Journal of Hand Surgery
525
526
The Journal of Hand Surgery / Vol. 28A No. 3 May 2003
Figure 2. Histopathology showing the cellular and vascular nature of the nodule rich with proliferation of collagen fibers. The fibroblasts were less numerous and aligned parallel to one another. The nodule contained markedly increased amounts of dense collagen fibers. Microscopic findings revealed no malignant findings.
Figure 1. A palpable lump (6 ⫻ 6 cm) in the palm. There was no cord-like thickening and no flexion contracture of either the metacarpophalangeal or proximal interphalangeal joints.
of the nodule, rich with proliferation of collagen fibers, and it was diagnosed as palmar fibromatosis grade II according to Luck’s3 classification (Fig. 2). Through a wide Bruner4 zigzag incision the giant fibrous nodule was dissected from the surrounding tissue and successfully excised. The nodule was found to exist beneath the neurovascular bundle and flexor tendons and extended from the base of the middle finger to the distal end of the transverse carpal ligament (Fig. 3). The tumor arose from the deepest longitudinal fibers that passed deep to either side of the flexor tendons. No pretendinous or digital cords were found during surgery.
Discussion Fibromatoses are a broad group of benign fibrous tissue proliferations of similar microscopic appearance that are intermediate in their biologic behavior between benign fibrous lesions and fibrosarcoma. The fibromatoses can be divided into 2 major groups. Superficial fibromatoses arise from the fascia or apo-
Figure 3. The giant palmar nodule (single arrow) revealed a round surface and was found to exist beneath the flexor tendons (double arrows) and neurovascular bundle (triple arrows) and extended from the base of the middle finger to the distal end of the transverse carpal ligament. The nodule was firm and scar-like on palpation and revealed a uniformly gray-white surface. The palmar aponeurosis was damaged partially by the giant nodule but no pretendinous or digital cords were found during surgery.
Muramatsu, Ihara, and Kawai / Giant Fibrous Nodule
neurosis and only rarely involve deeper structure. Deep fibromatosis (extra-abdominal desmoid) tend to be more aggressive in their behavior than the superficial fibromatoses and principally involve deeper structures, particularly the musculature of the trunk and the extremities.5 In this case, the tumor arose from the longitudinal fibers that passed deep to either side of the flexor tendons, we diagnosed this as palmar fibromatosis. Palmar fibromatosis belongs to the superficial fibromatoses group and its clinical course and progression are not uniform. McFarlane et al. divided the disease progression into 3 stages.6 – 8 In the classic scenario of typical disease a nodule develops in the palm and digits (stage I), followed by the formation of a pretendinous and digital cord that gradually contracts (stage II), leading to progressive digital flexion deformity (stage III). Although the origin of the palmar nodule is uncertain Rayan2 presented in his review that it seems to originate in the superficial components of the palmar fascia and its vertical fibers extend into the dermis. Apparently this patient’s presentation and progression was not compatible with that of typical Dupuytren’s disease. The giant palmar nodule was found to exist beneath the neurovascular bundle and between the flexor tendon and metacarpal bones. McGrouther divided the components of the palmer fascia into 3 layers.9 Layer 1 is the most superficial fibers inserting into the skin, layer 2 is the spiral fibers passing on either side of the flexor tendon, and layer 3 is the deepest longitudinal fibers passing deeply on either side of the flexor tendons and on either side of the metacarpal bones. In this case the
527
giant palmar nodule seemed to originate from McGrouther’s layer 3. The palmar nodule often regresses gradually giving way to development of a cord (stage I to II). The surgical findings revealed that the nodule grew like a soft-tissue tumor without development of any pretendinous cord or flexion deformity of the digits. Palmar fibrosis is uncommon in non-Caucasian races.10 In this case the patient had no risk factors such as diabetes mellitus, epilepsy, alcoholism, or trauma,11 and no ectopic fibromatosis was noted.
References 1. Rayan GM. Clinical presentation and types of Dupuytren’s disease. Hand Clin 1999;15:87–96. 2. Rayan GM. Palmar fascial complex anatomy and pathology in Dupuytren’s disease. Hand Clin 1999;15:73– 86. 3. Luck JV. Dupytren’s contracture. A new concept of the pathogenesis correlated with surgical management. J Bone Joint Surg 1959;41A:635– 664. 4. Bruner JM. The zig-zag volar-digital incision for flexortendon surgery. Plast Reconstr Surg 1967;40:571–574. 5. Enzinger FM. Fibromatosis. In: Enzinger FM, Weiss SW, eds. Soft tissue tumors. St. Louis: Mosby; 1994:201–230. 6. Caughell KA, McFarlane RM, McGrouther DA, Martin AH. Developmental anatomy of the palmar aponeurosis and its relationship to the palmaris longus tendon. J Hand Surg 1988;13A:485– 493. 7. McFarlane RM. Progress in Dupuytren’s disease [Editorial]. J Hand Surg 1991;16B:237–239. 8. McFarlane RM. The current status of Dupuytren’s disease. J Hand Surg 1983;8:703–708. 9. McGrouther DA. The microanatomy of Dupuytren’s contracture. Hand 1982;14:215–236. 10. Burge P. Genetics of Dupuytren’s disease. Hand Clin 1999; 15:63–71. 11. Ross DC. Epidemiology of Dupuytren’s disease. Hand Clin 1999;15:53– 62.