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Nodular fasciitis: A rapidly growing tumor of the hand
Nodular fasciitis: A rapidly growing tumor of the hand Nodular fasciitis is an uncommon benign neoplasm infrequently seen in the hand. There are often difficulties in diagnosis of this tumor. It is usually surgically excised while it is still small. The patient described here had a large and aggressive tumor that ruptured through the skin of the hand and extended to the periosteum of the ring metacarpal. (J HAND SURG 1991;16A: 791·5.)
Glenn Rankin, MD, Stuart H. Kuschner, MD, and Harris Gellman, MD,
Los Angeles, Calif.
Nodular fasciitis was first described in 1955 by Konwaler et al.' It is a benign reactive fibroblastic rapidly growing solitary subcutaneous lesion that before 1955 was probably mistaken for a malignant neoplasrn.i" The cause of the growth process is uncertain, although occasionally it is seen in areas of previous trauma." 3 It is most commonly found on the flexor aspect of the forearm."?
Case report E. A., a 39-year-old Hispanic woman, 2 weeks post partum noted a small painless mass in her left palm. She was seen in the Orthopaedic Hand Clinic at Los Angeles County + University of Southern California Medical Center approximately I month after the mass was first noted. She denied any history of trauma, infections, or any unusual activities involving the hand or the presence of any similar masses in the past, The examination of the hand at the first clinic visit revealed a I x 1 em mass, firm, nontender, and mobile in the hypothenar eminence of the left palm. Neurovascular examination results were normal with full finger range of motion. No lesions or marks were present on the skin of the hand. No epitrochlear or axillary lymph nodes were noted. Radiographs showed a soft tissue density over the fourth metacarpal From the Department of Orthopaedics, Division of Hand Surgery, Los Angeles County/University of Southern California Medical Center, Los Angeles, Calif. Received for publication Aug, 20, 1990; accepted in revised form Nov. 29, 1990. 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 aniele. Reprint requests: Stuart H. Kuschner, MD, 9001 Wilshire Blvd., Suite 200, Beverly Hills, CA 90211. 3/1127541
in the hypothenar eminence. No foreign body was seen, no calcification seen in the mass, and no periosteal reaction was seen along the underlying metacarpals. The diagnosis was unclear and the patient was scheduled to have magnetic resonance imaging (MRI) of the left hand, followed by an excisional biopsy. The patient returned to her home in Mexico for unspecified reasons before completing the scheduled test and surgery. While in Mexico she noted that the mass increased in size and eventually ruptured through the skin of the palm of her hand. She returned to the United States and was seen in the Orthopaedic Hand Clinic 5 months after her initial clinical evaluation. At this examination the patient was afebrile and in no apparent distress. The hand had a 4 ern x 3 cm x 3 ern firm, nonmobile, non-pulsatile, reddish-brown mass protruding out of the palm in the area of the hypothenar eminence of the left hand (Fig. 1). The skin edges were torn, jagged, and slightly erythematous. The wound had no active bleeding, and the mass glistened with a cover of serous fluid. The patient's hand had normal sensibility and capillary refill of all digits. The active arcs of motion of the long, ring, and small fingers of the left hand were limited by pain. No epitrochlear or axillary lymph nodes were noted. X-rays showed an increase in soft tissue density over the fourth metacarpal in the hypothenar eminence on the anterior to posterior view. No foreign body was seen. No calcifications were seen in the mass, and no periosteal reactions, lytic, or blastic changes were seen in the underlying metacarpals. The patient was admitted from the clinic to the hospital. Admission laboratory blood work was completely unremarkable. An angiogram and MRI of the left hand were done. The angiogram showed that the tumor was relatively hypervascularized and did not encroach on any major vascular structures. The MRI study showed that the mass extended down to the periosteum of the ring metacarpal (Fig. 2). A three-phase technetium 99m bone scan of the hand and her entire body showed a vascular blush in the tumor in the first phase of the scan; however, there was no evidence of uptake
THE JOURNALOF HAND SURGERY
791
792
The Journal of HAND SURGERY
Rankin et of.
A Fig. 1. A-B, Mass rupturing through the palmar skin.
Fig. 2. MRI showing the mass extending from the ring metacarpal.
Vol. 16A, No.5 September 199I
Nodular fasciitis
Fig. 3. Primary closure at wound edges after excision of mass.
Fig. 4. Low-power field composed of reactive appearing fibroblasts.
793
794
The Journal of HAND SURGERY
Rankin et al.
Fig. 5. High-power field, minimal cytologic atypia. in the bones of the hand or any of the other bones in her body. During the 3 days of testing, the mass enlarged by an additional centimeter in all dimensions. The patient had an excisional biopsy of the lesion. A tourniquet was used, but an elastic bandage was not used to exsanguinate the limb. The entire mass shelled out easily. The digital vessels, nerves, and flexor tendons had been pushed aside by the tumor and were all intact. The tumor was traced down to the periosteum of the ring metacarpal shaft on the flexor aspect and a piece of the periosteum was excised with the tumor. The wound was then closed primarily (Fig. 3). The tumor was a red and yellow round mass, 5 em x 4 em x 3 em. Microscopically the tumor consisted of plump loosely arranged immature fibroblasts in a dense reticulin meshwork with a small amount of birefringent collagen (Figs. 4 and 5). Areas of local increase in cellularity and few mitotic figures were seen. No evidence of metaplastic bone anywhere in the lesion was seen. A diagnosis of nodular fasciitis was assigned by the pathologist at our institution and was confirmed by Franz Enzinger, MD, Armed Forces Institute of Pathology. The patient remained in the hospital with 24 hours of intravenous antibiotics and was then discharged with a full range of motion and normal sensibility in all digits. The hand healed uneventfully, and the patient had no evidence of recurrence at the 10 month follow-up in the clinic.
Discussion Nodular fasciitis was first described in 1955 by Konwaler et aI. I and was initially called by the unwieldy
name of subcutaneous pseudosarcomatous fibromatosis. The entity was renamed pseudosarcomatous fasciitis in 1960 by Culberson and Enterline" and subsequently has been shortened to nodular fasciitis. It is a benign reactive fibroblastic tumor that before 1955 was probably mistaken for malignant neoplasms such as fibrosarcoma, angiosarcoma, liposarcoma, and malignant fibrous histiocytoma." 3. S It simulates the appearance of a sarcoma although it is not malignant, and it usually shows some inflammatory stigmata." The etiology of nodular fasciitis is uncertain although occasionally it is seen in areas of previous trauma." 3 The pathogenesis may involve a local reactive or inflammatory process of fibrous connective tissue. The tumor grows rapidly and patients are usually seen with a history of a mass for 1 to 2 weeks. The time from first detection to surgical excision is commonly less than 2 months.i" The average tumor size is 2 em x 2 em at excision. Nodular fasciitis usually presents as a rapid growing solitary subcutaneous lesion that occasionally involves the. muscle and the fascia.i" It has been reported in most all other tissues with the notable exception of the viscera. 2.3. S. 6 It is most commonly found in the forearm (27% to 29%), followed by the back and chest wall (15% to 18%) and the upper arm (12%).2.3, S, 7 The hands and feet are usually spared. 2-7 Approximately half of the patients have associated soreness, tenderness, or
Vol. 1M, No.5 September 1991
slight pain." 3.5.7 Numbness, paresthesia, or shooting pains are rare and result from the rapidly growing tumor exerting pressure on a peripheral nerve.' The lesion is seen about equally in males and females and is most commonly seen in young adults between the ages of 20 and 35 years.i": 7 The lesion is less common in infants and children and is rare in adults more than 60 years of age." 3. 5. 7 The gross findings of nodular fasciitis are not sufficiently distinctive to be identifiable from other tumors." 3. 7 It is usually a round to oval nodule that is not encapsulated and mayor may not be well circumscribed. Most commonly the tumors are tan to grayishwhite with a slimy consistency because of their myxoid character. The largest lesion is usually less than 2 'em; however, rare examples of larger tumors have been reported. 2.3.5.7 In the exceptionally large tumors, great care needs to be taken in making the diagnosis because other tumors, especially fibrosarcoma, can have areas that closely resemble nodular fasciitis.?: 3. 5.7 Microscopically all nodules consist predominantly of plump, immature appearing fibroblasts that are arranged in short irregular bundles and fascicles, accompanied by a dense reticulin meshwork with small amounts of mature collagen. Scattered lymphoid cells are intermixed with the fibroblasts and erythrocytes and occasionally lipid macrophages and multinucleated giant cells." 3. 7 Four important histologic features of nodular fasciitis are now recognized, including spindle-shaped fibroblasts, clefts separating fibroblasts, extravasated erythrocytes, and interstitial mucoid material.v" The intervening ground substance or matrix, which is made up primarily of mucopolysaccharides, is responsible for the loosely textured "feathery" pattern that is one of the most important criteria for diagnosis of nodular fasciitis." 3. 5. 7 Nodular fasciitis has caused many problems in determining diagnosis. In several studies nearly one half of the tumors were mistaken for a fibrosarcoma or some other type of malignant neoplasm." 3. 7 Many patients with nodular fasciitis have been treated by unnecessary and overly aggressive surgery.' Soft-tissue tumors that can present difficulty with distinction from nodular fasciitis include fibromatosis and fibrous histiocytoma. In fibromatosis, the lesion is usually well circumscribed and larger, and the cells are slender spindle-shaped fibroblasts that are arranged in long sweeping fascicles and are separated by abundant intercellular col-
Nodular fasciitis
795
lagen. Mitotic figures are seen in both but are more frequent in nodular fasciitis. Fibrous histiocytoma is less well circumscribed and is made up of more rounded, compact fibroblasts that are arranged in storiform or pinwheel pattern. In addition, fibrous histiocytoma has a well-organized vasculature, many lipid macrophages and siderophages, some giant cells, and a few mitoses." 3 . 7 Fibrosarcoma and malignant fibrous histiocytoma should be considered for larger and more cellular lesions. Microscopically, fibrosarcoma is characterized by densely packed cells, limited pleomorphism and hyperchromasia, and many mitotic figures. The cells are organized in interwoven fascicles that resemble a "herringbone" pattern. There is usually little or no interstitial collagen or mucoid material. Malignant fibrous histiocytoma is usually seen in an older population, and the cells have more individual cell pleomorphism.': 3. 7 Nodular fasciitis is a benign and usually self-limiting process that is well documented, although no cases of ulceration through the skin were found in the Iiterature.':? Nearly all nodules have been effectively treated by local excision. Recurrence is rare and occurs in 1% to 2% of all patients." 3. 7 When recurrence occurs it is soon after excision and is most likely the result of con. tinued growth after incomplete surgical removal of the lesion.' REFERENCES I. Konwaler BE, Keasbey L, Kaplan L. Subcutaneous pseudosarcomatous fibromatosis (fasciitis). A report of 8 cases. Am J Clin Pathol 1955;25:241-52. 2. Brimhall CL, Segura AD, McTigue MK, Easterly NB. Nodular fasciitis on the palm of a child. Arch Dermatol 1989;125:1441. 3. Enzinger FM, Weiss SW. Soft tissue tumors. 2nd ed. St. Louis: CV Mosby, 1988:103-13. 4. Shimizu S, Hashimoto H, Enjoji M. Nodular fasciitis: an analysis of 250 patients. Pathology 1984;16:161-6. 5. Stout AP. Pse~docarcomatous fasciitis in children. Cancer 1961;14:1216-22. 6. Allen PW. Nodular fasciitis. Pathology 1972;4:9-26. 7. Bernstein KE, Lattes R. Nodular (pseudosarcomatous) fasciitis, a nonrecurrent lesion: clinicopathologic study of 134 cases. Cancer 1982;49:1668-78. 8. Culberson JD, Enterline HT. Pseudosarcomatous fasciitis. A distinctive clinical-pathologic entity: a report of five cases. Ann Surg 1960;151:235-40.
Glenn Rankin, MD, Stuart H. Kuschner, MD, and Harris Gellman, MD,
Los Angeles, Calif.
Nodular fasciitis was first described in 1955 by Konwaler et al.' It is a benign reactive fibroblastic rapidly growing solitary subcutaneous lesion that before 1955 was probably mistaken for a malignant neoplasrn.i" The cause of the growth process is uncertain, although occasionally it is seen in areas of previous trauma." 3 It is most commonly found on the flexor aspect of the forearm."?
Case report E. A., a 39-year-old Hispanic woman, 2 weeks post partum noted a small painless mass in her left palm. She was seen in the Orthopaedic Hand Clinic at Los Angeles County + University of Southern California Medical Center approximately I month after the mass was first noted. She denied any history of trauma, infections, or any unusual activities involving the hand or the presence of any similar masses in the past, The examination of the hand at the first clinic visit revealed a I x 1 em mass, firm, nontender, and mobile in the hypothenar eminence of the left palm. Neurovascular examination results were normal with full finger range of motion. No lesions or marks were present on the skin of the hand. No epitrochlear or axillary lymph nodes were noted. Radiographs showed a soft tissue density over the fourth metacarpal From the Department of Orthopaedics, Division of Hand Surgery, Los Angeles County/University of Southern California Medical Center, Los Angeles, Calif. Received for publication Aug, 20, 1990; accepted in revised form Nov. 29, 1990. 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 aniele. Reprint requests: Stuart H. Kuschner, MD, 9001 Wilshire Blvd., Suite 200, Beverly Hills, CA 90211. 3/1127541
in the hypothenar eminence. No foreign body was seen, no calcification seen in the mass, and no periosteal reaction was seen along the underlying metacarpals. The diagnosis was unclear and the patient was scheduled to have magnetic resonance imaging (MRI) of the left hand, followed by an excisional biopsy. The patient returned to her home in Mexico for unspecified reasons before completing the scheduled test and surgery. While in Mexico she noted that the mass increased in size and eventually ruptured through the skin of the palm of her hand. She returned to the United States and was seen in the Orthopaedic Hand Clinic 5 months after her initial clinical evaluation. At this examination the patient was afebrile and in no apparent distress. The hand had a 4 ern x 3 cm x 3 ern firm, nonmobile, non-pulsatile, reddish-brown mass protruding out of the palm in the area of the hypothenar eminence of the left hand (Fig. 1). The skin edges were torn, jagged, and slightly erythematous. The wound had no active bleeding, and the mass glistened with a cover of serous fluid. The patient's hand had normal sensibility and capillary refill of all digits. The active arcs of motion of the long, ring, and small fingers of the left hand were limited by pain. No epitrochlear or axillary lymph nodes were noted. X-rays showed an increase in soft tissue density over the fourth metacarpal in the hypothenar eminence on the anterior to posterior view. No foreign body was seen. No calcifications were seen in the mass, and no periosteal reactions, lytic, or blastic changes were seen in the underlying metacarpals. The patient was admitted from the clinic to the hospital. Admission laboratory blood work was completely unremarkable. An angiogram and MRI of the left hand were done. The angiogram showed that the tumor was relatively hypervascularized and did not encroach on any major vascular structures. The MRI study showed that the mass extended down to the periosteum of the ring metacarpal (Fig. 2). A three-phase technetium 99m bone scan of the hand and her entire body showed a vascular blush in the tumor in the first phase of the scan; however, there was no evidence of uptake
THE JOURNALOF HAND SURGERY
791
792
The Journal of HAND SURGERY
Rankin et of.
A Fig. 1. A-B, Mass rupturing through the palmar skin.
Fig. 2. MRI showing the mass extending from the ring metacarpal.
Vol. 16A, No.5 September 199I
Nodular fasciitis
Fig. 3. Primary closure at wound edges after excision of mass.
Fig. 4. Low-power field composed of reactive appearing fibroblasts.
793
794
The Journal of HAND SURGERY
Rankin et al.
Fig. 5. High-power field, minimal cytologic atypia. in the bones of the hand or any of the other bones in her body. During the 3 days of testing, the mass enlarged by an additional centimeter in all dimensions. The patient had an excisional biopsy of the lesion. A tourniquet was used, but an elastic bandage was not used to exsanguinate the limb. The entire mass shelled out easily. The digital vessels, nerves, and flexor tendons had been pushed aside by the tumor and were all intact. The tumor was traced down to the periosteum of the ring metacarpal shaft on the flexor aspect and a piece of the periosteum was excised with the tumor. The wound was then closed primarily (Fig. 3). The tumor was a red and yellow round mass, 5 em x 4 em x 3 em. Microscopically the tumor consisted of plump loosely arranged immature fibroblasts in a dense reticulin meshwork with a small amount of birefringent collagen (Figs. 4 and 5). Areas of local increase in cellularity and few mitotic figures were seen. No evidence of metaplastic bone anywhere in the lesion was seen. A diagnosis of nodular fasciitis was assigned by the pathologist at our institution and was confirmed by Franz Enzinger, MD, Armed Forces Institute of Pathology. The patient remained in the hospital with 24 hours of intravenous antibiotics and was then discharged with a full range of motion and normal sensibility in all digits. The hand healed uneventfully, and the patient had no evidence of recurrence at the 10 month follow-up in the clinic.
Discussion Nodular fasciitis was first described in 1955 by Konwaler et aI. I and was initially called by the unwieldy
name of subcutaneous pseudosarcomatous fibromatosis. The entity was renamed pseudosarcomatous fasciitis in 1960 by Culberson and Enterline" and subsequently has been shortened to nodular fasciitis. It is a benign reactive fibroblastic tumor that before 1955 was probably mistaken for malignant neoplasms such as fibrosarcoma, angiosarcoma, liposarcoma, and malignant fibrous histiocytoma." 3. S It simulates the appearance of a sarcoma although it is not malignant, and it usually shows some inflammatory stigmata." The etiology of nodular fasciitis is uncertain although occasionally it is seen in areas of previous trauma." 3 The pathogenesis may involve a local reactive or inflammatory process of fibrous connective tissue. The tumor grows rapidly and patients are usually seen with a history of a mass for 1 to 2 weeks. The time from first detection to surgical excision is commonly less than 2 months.i" The average tumor size is 2 em x 2 em at excision. Nodular fasciitis usually presents as a rapid growing solitary subcutaneous lesion that occasionally involves the. muscle and the fascia.i" It has been reported in most all other tissues with the notable exception of the viscera. 2.3. S. 6 It is most commonly found in the forearm (27% to 29%), followed by the back and chest wall (15% to 18%) and the upper arm (12%).2.3, S, 7 The hands and feet are usually spared. 2-7 Approximately half of the patients have associated soreness, tenderness, or
Vol. 1M, No.5 September 1991
slight pain." 3.5.7 Numbness, paresthesia, or shooting pains are rare and result from the rapidly growing tumor exerting pressure on a peripheral nerve.' The lesion is seen about equally in males and females and is most commonly seen in young adults between the ages of 20 and 35 years.i": 7 The lesion is less common in infants and children and is rare in adults more than 60 years of age." 3. 5. 7 The gross findings of nodular fasciitis are not sufficiently distinctive to be identifiable from other tumors." 3. 7 It is usually a round to oval nodule that is not encapsulated and mayor may not be well circumscribed. Most commonly the tumors are tan to grayishwhite with a slimy consistency because of their myxoid character. The largest lesion is usually less than 2 'em; however, rare examples of larger tumors have been reported. 2.3.5.7 In the exceptionally large tumors, great care needs to be taken in making the diagnosis because other tumors, especially fibrosarcoma, can have areas that closely resemble nodular fasciitis.?: 3. 5.7 Microscopically all nodules consist predominantly of plump, immature appearing fibroblasts that are arranged in short irregular bundles and fascicles, accompanied by a dense reticulin meshwork with small amounts of mature collagen. Scattered lymphoid cells are intermixed with the fibroblasts and erythrocytes and occasionally lipid macrophages and multinucleated giant cells." 3. 7 Four important histologic features of nodular fasciitis are now recognized, including spindle-shaped fibroblasts, clefts separating fibroblasts, extravasated erythrocytes, and interstitial mucoid material.v" The intervening ground substance or matrix, which is made up primarily of mucopolysaccharides, is responsible for the loosely textured "feathery" pattern that is one of the most important criteria for diagnosis of nodular fasciitis." 3. 5. 7 Nodular fasciitis has caused many problems in determining diagnosis. In several studies nearly one half of the tumors were mistaken for a fibrosarcoma or some other type of malignant neoplasm." 3. 7 Many patients with nodular fasciitis have been treated by unnecessary and overly aggressive surgery.' Soft-tissue tumors that can present difficulty with distinction from nodular fasciitis include fibromatosis and fibrous histiocytoma. In fibromatosis, the lesion is usually well circumscribed and larger, and the cells are slender spindle-shaped fibroblasts that are arranged in long sweeping fascicles and are separated by abundant intercellular col-
Nodular fasciitis
795
lagen. Mitotic figures are seen in both but are more frequent in nodular fasciitis. Fibrous histiocytoma is less well circumscribed and is made up of more rounded, compact fibroblasts that are arranged in storiform or pinwheel pattern. In addition, fibrous histiocytoma has a well-organized vasculature, many lipid macrophages and siderophages, some giant cells, and a few mitoses." 3 . 7 Fibrosarcoma and malignant fibrous histiocytoma should be considered for larger and more cellular lesions. Microscopically, fibrosarcoma is characterized by densely packed cells, limited pleomorphism and hyperchromasia, and many mitotic figures. The cells are organized in interwoven fascicles that resemble a "herringbone" pattern. There is usually little or no interstitial collagen or mucoid material. Malignant fibrous histiocytoma is usually seen in an older population, and the cells have more individual cell pleomorphism.': 3. 7 Nodular fasciitis is a benign and usually self-limiting process that is well documented, although no cases of ulceration through the skin were found in the Iiterature.':? Nearly all nodules have been effectively treated by local excision. Recurrence is rare and occurs in 1% to 2% of all patients." 3. 7 When recurrence occurs it is soon after excision and is most likely the result of con. tinued growth after incomplete surgical removal of the lesion.' REFERENCES I. Konwaler BE, Keasbey L, Kaplan L. Subcutaneous pseudosarcomatous fibromatosis (fasciitis). A report of 8 cases. Am J Clin Pathol 1955;25:241-52. 2. Brimhall CL, Segura AD, McTigue MK, Easterly NB. Nodular fasciitis on the palm of a child. Arch Dermatol 1989;125:1441. 3. Enzinger FM, Weiss SW. Soft tissue tumors. 2nd ed. St. Louis: CV Mosby, 1988:103-13. 4. Shimizu S, Hashimoto H, Enjoji M. Nodular fasciitis: an analysis of 250 patients. Pathology 1984;16:161-6. 5. Stout AP. Pse~docarcomatous fasciitis in children. Cancer 1961;14:1216-22. 6. Allen PW. Nodular fasciitis. Pathology 1972;4:9-26. 7. Bernstein KE, Lattes R. Nodular (pseudosarcomatous) fasciitis, a nonrecurrent lesion: clinicopathologic study of 134 cases. Cancer 1982;49:1668-78. 8. Culberson JD, Enterline HT. Pseudosarcomatous fasciitis. A distinctive clinical-pathologic entity: a report of five cases. Ann Surg 1960;151:235-40.