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The operative treatment of malignant tumors and aggressive benign tumors involving the distal end of the ulna often necessitates en bloc resection. The oncological and functional results for eight patients in whom a neoplasm involving the distal end of the ulna had been treated with en bloc resection without reconstruction of the osseous defect were reviewed retrospectively at a mean of seventy-nine months (range, twenty-three to 271 months). Four patients had a giant-cell tumor; two, a low-grade osteogenic sarcoma; one, a hemangioendothelioma; and one, a soft-tissue epithelioid sarcoma with osseous involvement. The amount of bone that was removed from the distal end of the ulna ranged from 3.1 to 9.0 centimeters. In the four patients who had a malignant tumor, a minimum of 7.5 centimeters was removed in order to achieve an adequate wide margin proximally. In the patients who had a benign tumor, a maximum of 6.6 centimeters was resected. Extraperiosteal resection was performed in three of the patients who had a malignant tumor and in one of the patients who had an aggressive giant-cell tumor. Subperiosteal resection was performed in the three patients who had a benign tumor and in one patient who had a parosteal osteogenic sarcoma. None of the patients had local or systemic evidence of recurrence of the tumor. The functional result was excellent for six patients and good for two. Grip strength was reduced by a mean of 15 per cent compared with the strength on the contralateral side, and this reduction did not appear to be related directly to the amount of bone that had been resected. The findings of this study support the concept that routine reconstruction of the osseous defect is not necessary after en bloc resection of a neoplasm of the distal end of the ulna.
The operative treatment of tumors involving the distal end of the ulna may require en bloc resection of the distal end of the ulna. The resultant absence of the bone and soft-tissue support of the ulnar side of the distal part of the forearm and carpus has led some to suggest the need for reconstruction of the osseous defect15,27. To determine whether this reconstruction is necessary, we reviewed our experience and evaluated the functional result for eight patients who had been managed with an en bloc resection, without reconstruction, for an aggressive benign or a malignant tumor of the distal end of the ulna.
Through a retrospective review of the operative and clinical records for the years 1970 to 1993 at the Mayo Clinic, we identified eight patients who had been managed with isolated en bloc resection for a malignant tumor or an aggressive benign tumor of the distal end of the ulna (Table I). No patient was excluded from the study for any reason.
Six patients were men and two were women; the mean age at the time of the resection was thirty-four years (range, nineteen to fifty years). The right limb was dominant in the seven patients (Cases 1, 2, and 4 through 8) for whom this information was known; the dominant extremity was involved in four of these patients. In seven patients, the tumor originated in the distal end of the ulna. Four of these patients had a giant-cell tumor; one, a grade-1 parosteal osteogenic sarcoma; one, a grade-2 parosteal osteogenic sarcoma; and one, a grade-1 hemangioendothelioma (Table I). The remaining patient had an epithelioid sarcoma with osseous involvement. The presenting symptom was pain in six patients and a soft-tissue swelling associated with a soft-tissue tumor in one patient (Case 1). The remaining patient (Case 8) was seen because of decreased grip strength, local tenderness, and a tingling sensation in the distribution of the ulnar nerve eight years after apparent healing of a fracture of the distal end of the ulna. Three patients (Cases 1, 3, and 8) had had an operative procedure before they were seen at our institution. One patient (Case 1) had had a biopsy, which had showed evidence of an epithelioid sarcoma. Curettage and bone-grafting had been performed in another patient (Case 3), and the operative specimens had demonstrated a hemangioendothelioma. The third patient (Case 8) had been managed with two previous operations for a parosteal osteogenic sarcoma. The first operative procedure involved removal of what was thought to be an osteochondroma with an associated ganglion cyst, which was believed to be post-traumatic and contributing to ulnar neuropathy. No histological findings from the operative specimens were reported, but after re-excision for local recurrence one month later histological analysis suggested a grade-1 parosteal osteogenic sarcoma. This classification was revised to grade 2 after histological examination of the specimen resected at the index operation. Staging of the tumors was done according to the classification system of Enneking; three of the giant-cell tumors were classified as aggressive on the basis of the increasing size, an associated soft-tissue mass, pain, or a radiographic appearance of a ragged permeative interface with adjacent bone, cortical destruction, or soft-tissue extension. The fourth giant-cell tumor was seen to have become enlarged radiographically and was moderately symptomatic, but it lacked any other clinical or radiographic features suggestive of aggressive behavior. When possible (in Cases 2, 3, and 8), the histological features of the malignant tumors were classified according to the method of Broders et al., who assigned one of four grades on the basis of cellularity, invasiveness, and mitotic activity. All of the malignant tumors were considered to be low grade (grade 1 or 2) on the basis of their histological appearance and to be associated with a low risk of distant metastasis and an indolent rate of evolution. In the patient (Case 1) who had the epithelioid sarcoma, the tumor involved bone, had extended beyond its soft-tissue compartment, and was classified as stage IB according to the system of Enneking. In the patient (Case 3) who had the hemangioendothelioma of bone, the tumor extended beyond the confines of the bone and was also classified as stage IB. The patient (Case 8) in whom a previous operation for a grade-2 osteogenic sarcoma had been inadequate, with soft-tissue contamination, was considered to have a stage-IB tumor. In the patient (Case 2) who had the grade-1 osteogenic sarcoma, the tumor appeared to be entirely within the ulna and was classified as stage IA. The local staging of the malignant tumors was based on the findings on magnetic resonance images as well as radiographs (Cases 1 and 5) or the findings on computed tomography scans (Case 4). Pulmonary metastases were not found on radiographs or, in some patients, computed tomography scans of the chest. In one patient (Case 1), a bone scan with technetium-99m methylene diphosphonate showed increased uptake locally but no distant involvement. No patient was found initially to have distant metastases. The operative technique varied according to the histological nature of the tumor, the location of any previous biopsy incision, and the extent of osseous involvement. The level of resection of the ulna was determined by the extent of osseous involvement, as seen on the preoperative imaging studies. An additional two centimeters of radiographically normal-appearing bone was resected with each malignant tumor in order to achieve a wide margin (a plane of dissection beyond the reactive zone and through normal tissue12). Only enough normal-appearing bone was resected with each benign tumor to maintain a barrier of normal tissue between the edge of the lesion and the margin of resection, again accomplishing a wide margin. A wide margin was achieved in all but two patients: one (Case 2) who had a marginal excision of a grade-1 parosteal osteosarcoma and one (Case 7) who had a contaminated marginal excision of a giant-cell tumor. A marginal margin is defined as an extracapsular plane of dissection within the reactive zone12. A marginal excision is considered to have become contaminated when a final marginal margin was accomplished but only after the tumor had been entered. No additional operative resection was performed in either of these two patients. The amount of bone removed from the distal end of the ulna ranged from 3.1 to 9.0 centimeters. A minimum of 7.5 centimeters was removed in all of the patients who had a malignant tumor, in order to achieve a wide margin proximally. A maximum of 6.6 centimeters was resected in the patients who had a benign tumor. Subperiosteal resection was performed in three of the four patients who had a benign tumor, and extraperiosteal resection was done in three of the four patients who had a malignant tumor. The exceptions were one patient (Case 6) who had an extraperiosteal resection of an aggressive giant-cell tumor and one (Case 2) who had a subperiosteal resection of a grade-1 parosteal osteogenic sarcoma. The soft-tissue extension of the giant-cell tumor (Case 6) was thought to warrant extraperiosteal resection so that a wide margin could be achieved. In the other exceptional patient (Case 2), the subperiosteal resection was done, accomplishing a marginal margin12, for what was discovered on final histological examination to be a grade-1 parosteal osteogenic sarcoma. After subperiosteal resection, the periosteal sleeve was closed meticulously to allow radial deviation to neutral while preventing radial translation of the carpus relative to the distal radial articular surface. The triangular fibrocartilage proper was repaired with suture to the ulnar collateral ligament, the dorsal radio-ulnar ligament, the volar radio-ulnar ligament, and, when additional anchorage was deemed necessary by the operating surgeon, the sheath of the extensor carpi ulnaris. Extraperiosteal dissection, which included resection of the triangular fibrocartilage complex, the ulnar border of the pronator quadratus, and the distal radio-ulnar joint capsule, was performed in all of the patients who had a malignant tumor (except Case 2) and in one patient (Case 6) who had an aggressive giant-cell tumor, in order to obtain an acceptable wide margin distally and circumferentially. In the patient who had the soft-tissue sarcoma (Case 1), the extensor carpi ulnaris was resected with the tumor. In two patients who had a malignant tumor (Cases 1 and 8), the dorsal sensory branch of the ulnar nerve was sacrificed with the resected specimen. The nerve branches were resected proximally, and the cut end was allowed to retract within the muscle to minimize symptoms from the subsequent neuroma. Postoperatively, all patients had immobilization of the extremity in an ulnar-gutter below-the-elbow splint, with the forearm in neutral, for a minimum of two weeks. Only one patient (Case 1) had adjunctive treatment, which included brachytherapy (at a dose of fifteen gray) and external-beam radiation therapy (at a dose of fifteen gray) postoperatively. Brachytherapy is the implantation of radioactive material at the site of the tumor. For patients who have a soft-tissue tumor in an extremity (such as our patient), the radioactive material, in the form of pellets, is introduced through tubes that are inserted, at the time of the operation, through the skin adjacent to the incision and placed next to the tumor bed22. After completion of the radiation therapy, the patient had a transfer of a latissimus dorsi free flap to restore bulk to the ulnar side of the forearm, split-thickness skin-grafting to cover the flap, and a tendon transfer of the extensor carpi radialis longus to the extensor carpi ulnaris. The extensor carpi ulnaris tendon had been resected at the time of the initial operation because of tumor involvement. Each patient was re-examined by an orthopaedic oncologist and a hand surgeon in our clinic at least once after the operation, and six patients (Cases 1, 2, 3, 5, 7, and 8) were examined by a hand surgeon in our clinic at the most recent follow-up visit. At the time of follow-up, the hand surgeon examined each patient specifically to assess the stability of the radiocarpal joint and the ulnar stump. To determine the stability of the ulnar stump, the surgeon checked for movement of the stump relative to the stabilized distal end of the radius. To determine radiocarpal stability, the surgeon attempted volar-dorsal and radio-ulnar translation of the hand and carpus relative to the stabilized distal end of the radius and compared the finding with that on the contralateral side. No examination for instability was performed for the two patients (Cases 4 and 6) who were seen by a local orthopaedic surgeon rather than at our institution. Initially, the range of motion and the grip strength were measured by the nursing staff at the Mayo Hand Surgery Clinic and then by orthopaedic surgeons at that clinic. The range of motion and the grip strength at the time of the latest follow-up were assessed in the Mayo Hand Surgery Clinic (six patients) or by a local orthopaedic surgeon (two patients). A standard goniometer was used to measure the range of motion, which was recorded numerically, for six patients. A Jamar dynamometer (Therapeutic Instruments, Clifton, New Jersey) was used to measure the grip strength of all eight patients; a 10 per cent conversion factor was used to adjust the measurement for limb dominance. Additional clinical follow-up information was obtained by means of a telephone conversation for some patients. The oncological result at the time of follow-up was assessed in terms of the margin achieved intraoperatively, the presence or absence of local recurrence, and, for the patients who had a malignant tumor, evidence of systemic metastasis on radiographs and computed tomography scans of the chest. The functional result was graded as excellent, good, fair, or poor. The result was excellent when there was no pain, no instability of the carpus relative to the distal radial articular surface, no instability of the distal part of the remaining ulnar diaphysis, no noticeable side-to-side difference in grip strength according to the patient, and no restriction of activities. The result was considered good when the patient had occasional pain, mild restriction of activities, occasional clicking of the distal part of the remaining ulnar diaphysis, mildly decreased grip strength, and no radiocarpal instability. A fair result was defined as moderate pain that necessitated the occasional use of pain medication, modifications of functionally important activities, frequent clicking of the distal part of the remaining ulnar diaphysis, decreased grip strength, or occasional radiocarpal instability. A poor result was defined as severe pain that necessitated the regular use of pain medication or that caused vocational limitations, severely decreased grip strength, or more severe radiocarpal instability.
The mean interval between the operation and the latest clinical examination was seventy-nine months (range, twenty-three to 271 months), the mean interval between the operation and the most recent clinical examination at our clinic was thirty-two months, and the mean interval between the operation and the objective assessment of the grip strength and the range of motion was thirty-six months. At the time of the most recent follow-up visit, the patients were employed as an analytical chemist (Case 1), a mechanic (Case 2), a radiography technician (Case 3), an internal medicine physician (Case 4), a lawyer (Case 5), a sewing-machine repairman (Case 6), a veterinary technician (Case 7), and a domestic worker (Case 8). Only two patients (Cases 6 and 7) had changed their occupation since the time of the initial operation; one (Case 6) had been a retailer and the other (Case 7) had been a student. Specific recreational interests at the time of follow-up included weight-lifting (Case 1), jig-fishing (Case 4), and basketball (Case 7). The oncological result was excellent for all patients. After the options had been discussed with each of the two patients who had had less than a wide margin, both chose to be observed for local recurrence rather than to have additional resection. No recurrences were found in any of the eight patients. The four patients who had had a sarcoma had no evidence of metastasis on follow-up radiographs or computed tomography scans of the chest that were made at a mean of 110 months postoperatively. The functional result was good or excellent for all patients (Figs. 1-A and 1-B). None of the eight patients had so-called clunking or laxity, suggestive of radiocarpal instability, and none had a limited range of motion. No patient had radiographic evidence of ulnocarpal translocation. Six patients rated the grip strength as excellent; one, as adequate; and one, as diminished. Six patients had no pain or limitation of activity. One patient had occasional pain when lifting heavy objects above the head but was otherwise asymptomatic. One patient who had a good subjective result was a forty-eight-year-old internal medicine physician (Case 4) who had had resection of a giant-cell tumor. Forty-five months postoperatively, he reported a loss of grip strength and some pain when gripping pliers, but there was no clunking or laxity of the wrist. The patient also noted some clicking of the distal end of the ulna associated with mild pain when he used the hand to cast a fishing rod. The second patient (Case 1) who had only a good result was a twenty-six-year-old chemist who rated the grip strength as only adequate fifty months postoperatively and was not satisfied with the cosmetic appearance after a wide resection of an epithelioid sarcoma, despite a tendon transfer and the use of a free flap (Figs. 2-A, 2-B, 2-C through 2-D). He had no pain or limitations of activity.
Objective testing of grip strength and the range of motion confirmed the satisfactory over-all functional results described by the patients. The grip strength on the involved side was decreased by a maximum of 38 per cent (32 per cent after adjustment for limb dominance) compared with that on the contralateral side (twenty-six compared with forty-two pounds [11.8 compared with 19.1 kilograms]). The grip strength was decreased by a mean of 15 per cent (19 per cent after adjustment for dominance) compared with that on the contralateral side. The grip strength on the involved side ranged from sixteen pounds (7.3 kilograms), compared with twenty-four pounds (10.9 kilograms) on the contralateral side, to 125 pounds (56.7 kilograms), which was equal to the strength on the contralateral side. After adjustment for dominance, the grip strength on the involved side ranged from 17.6 to 112.5 pounds (8.0 to 51.0 kilograms). The reduction in grip strength did not appear to be related directly to the amount of bone that had been resected: one patient (Case 8) in whom 9.0 centimeters of bone had been resected had only a 13 per cent reduction in grip strength, and the patient (Case 1) in whom 7.8 centimeters of bone had been resected had the greatest disparity (38 per cent) postoperatively. The range of motion was documented in detail for six patients. Extension of the wrist averaged 66.7 degrees (range, 45 to 85 degrees), compared with 73.3 degrees (range, 65 to 90 degrees) on the uninvolved side; flexion of the wrist averaged 58.3 degrees (range, 25 to 85 degrees), compared with 73.3 degrees (range, 60 to 90 degrees) on the uninvolved side; radial and ulnar deviation averaged 21.7 degrees (range, 10 to 35 degrees) and 30.8 degrees (range, 0 to 55 degrees), respectively, compared with 23.3 degrees (range, 15 to 35 degrees) and 33.3 degrees (range, 0 to 50 degrees); and pronation of the forearm averaged 80.0 degrees (range, 60 to 90 degrees), compared with 82.5 degrees (range, 75 to 90 degrees). Supination of the forearm was essentially the same on both sides (mean, 85.0 degrees; range, 80 to 90 degrees). The remaining two patients were reported to have a full range of motion of both the forearm and the wrist, compared with the motion on the contralateral side, but specific measurements were not documented. Extension on the involved side was 91 per cent that on the contralateral side; flexion, 80 per cent; radial deviation, 93 per cent; ulnar deviation, 93 per cent; pronation, 97 per cent; and supination, 100 per cent. Hypoesthesia in the distribution of the dorsal sensory branch of the ulnar nerve was found only in the two patients (Cases 1 and 8) in whom this branch was intentionally sacrificed during a wide excision of a sarcoma. The patient (Case 1) who had had a wide excision of a soft-tissue sarcoma, postoperative irradiation, transfer of a latissimus dorsi free flap, and tendon transfer had an over-all rating of good because of the subjective decrease in grip strength and the objective finding of a markedly limited range of motion. The reduction in grip strength (twenty-six compared with forty-two pounds [11.8 compared with 19.1 kilograms]) was confirmed by testing with a dynamometer. There were no other complications.
The distal end of the ulna is an uncommon site for a tumor. The tumors most frequently reported to develop in that area include intraosseous ganglia13,25,28 and osteochondromas4,8. In a few case reports, metastatic tumors7,15, synovial chondromatosis18, fibromatosis of bone9, desmoplastic fibromas27, solitary bone cysts in children26, traumatic epidermoid inclusion cysts19, and giant-cell tumors17 have been described. Other tumors of the distal end of the ulna have been included in larger series of tumors with similar histogenesis, but they usually account for only a small percentage of all tumors of a given type. In the experience at the Memorial Sloan-Kettering Cancer Center16, three of 265 giant-cell tumors, one of 1095 osteogenic sarcomas, eight of 334 Ewing sarcomas, and three non-Hodgkin lymphomas of 158 bone lymphomas were located in the distal part of the ulna and twelve of 364 osteoblastomas, eight of 323 osteochondromas, and eight of 394 aneurysmal bone cysts were found in the distal end of either the radius or the ulna. To our knowledge, we are the first to report on a series of tumors in the distal end of the ulna that were treated with en bloc resection. Functionally, the distal end of the ulna aids in both rotation of the forearm and grip strength as well as in maintaining the relationship between the carpus and the distal end of the radius. The ulnar collateral ligament of the wrist, which emanates from the ulnar styloid process, and the triangular fibrocartilage complex play important roles in the maintenance of this anatomical relationship23,24. The classic Darrach procedure has been used primarily for patients who have post-traumatic or degenerative osteoarthrosis or rheumatoid arthritis of the distal radio-ulnar joint5. With that procedure, the distal end of the ulna is approached through the interval between the flexor carpi ulnaris and the extensor carpi ulnaris, preserving the dorsal cutaneous branch of the ulnar nerve. Subperiosteal dissection is performed, and a resection of the distal three centimeters of the ulna (or to the proximal extent of the sigmoid notch) is carried out. The tip of the ulnar styloid process is preserved with its attachment to the ulnar collateral ligament. The periosteum is closed in a sleeve to prevent laxity from developing ulnarly. When the Darrach procedure is performed to treat a neoplasm of the distal end of the ulna, the approach, type of dissection (subperiosteal or extraperiosteal), and degree of ulnar resection are dictated by the type, behavior, and anatomical extent of the tumor. Potential problems with the Darrach procedure or resection of a longer segment of the distal part of the ulna include destruction of the osseous support for the triangular fibrocartilage complex, ulnocarpal instability, unstable rotation of the carpal unit around the ulnar axis, and abutment of the distal part of the remaining ulnar diaphysis against the radius causing painful snapping or tendinous rupture1-3,5,14,20,21. The technical measures that have been suggested to minimize these problems include resection of only as much bone as is necessary to accomplish the purpose of the procedure, preservation of the styloid process, and periosteal closure after subperiosteal dissection11. When the distal end of the ulna is resected for an oncological reason, the primary goal of excision of the tumor takes precedence over the restoration of function. Two unsubstantiated criticisms of the Darrach procedure have been ulnocarpal translocation and decreased grip strength5. None of the patients in the present series had ulnocarpal translocation. All patients lost grip strength but usually to only a minor degree. In this small group of patients, the reduction in grip strength was not related to the amount of bone that had been resected. There is no clear explanation as to why the problems that have been reported after the less extensive Darrach procedure did not develop to the same degree in our patients. In some case reports of neoplasms of the distal end of the ulna that were treated with en bloc resection, the defect was reconstructed15,27. On the basis of our experience with the eight patients reported on here, we do not believe that such reconstruction is necessary. Reconstruction with bone allograft, cement, a metallic prosthesis, and even autogenous bone graft adds unnecessary risk and morbidity without demonstrable functional gain. All eight of our patients had an excellent or good functional result without reconstruction and regardless of the extent of the resection. None of our patients had a previous fracture of the wrist, ligamentous laxity, or rheumatoid arthritis.
*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. No funds were received in support of this study.
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