John C. Ivins

Malignant fibrous histiocytoma: a retrospective study of 167 cases.

A retrospective study of 167 patients with soft‐tissue malignant fibrous histiocytoma of the trunk, extremities, and retroperitoneal region revealed twice as many deeply situated tumors as superficial tumors. Malignant fibrous histiocytoma may be subclassified into fibrous, giant‐cell, myxoid, and inflammatory variants. The fibrous variant accounted for two‐thirds of the lesions. The prognosis is no different among the histologic subtypes. The depth of the tumor significantly affects survival, and three important groups were identified: superficial tumors, superficial tumors that recur in deep locations, and deeply situated tumors. The group with superficial tumors that subsequently did not recur in deep locations had a significantly better 4‐year survival rate than did the other two groups (65% versus 34% and 40%, respectively). Patients with distally located tumors had a better 5‐year survival rate than did patients with proximally located tumors (73% versus 28%). Local recurrence was found in 51% of patients who had a “complete” excision. Patients with superficial tumors had a higher local recurrence rate (71%) than did those with deep tumors (41%). Few patients with retroperitoneal tumors were long‐term survivors; the 5‐year survival rate was 14%.

A prospective randomized study of the efficacy of routine elective lymphadenectomy in management of malignant melanoma. Preliminary results

To determine whether immediate or delayed lymphadenectomy is more beneficial than none in cases of localized (stage I) melanoma, we undertook in 1972 a prospective randomized study. Patients with midline trunk lesions were excluded as well as patients with lesions situated directly over the node‐bearing area. In addition, because of the low risk of metastasis, the protocol was changed to exclude level 2 lesions. Of the 173 patients studied, 63 were randomized to no lymphadenectomy, 56 to delayed (3 months) lymphadenectomy, and 54 to immediate lymphadenectomy. None of these regimens differed significantly from the others in its effect on length of survival or interval to metastasis. And of the 110 patients who underwent elective lymphadenectomy, 103 were without nodal involvement. Our preliminary conclusion is that elective node dissection is not beneficial in management of melanoma. However, disease progression was advanced significantly by age of the patient (>60 years) and by invasiveness (level 4 or 5) and thickness (>1.5 mm) of the melanoma.

Benign chondroblastoma.A study of 125 cases

Chondroblastoma is a benign bone tumor arising most often in the epiphyses of long bones. Nearly 90% occurred in patients between the ages of 5 and 25 years, but tumors were found in later adulthood and even in old age. A few chondroblastomas were predominantly cystic, and some contained zones that were indistinguishable microscopically from aneurysmal bone cysts. Conservative surgical removal, nearly always effective, should be thorough so as to minimize the risk of the occasional troublesome recurrence. Late sarcomatous change has been reported in a few cases whether radiation therapy was employed or not. Atypical sarcomas, especially chondrosarcomas, mimic chondroblastoma in rare instances, but there is no convincing evidence for a primarily malignant chondroblastoma. The one metastasizing benign chondroblastoma that was documented is a pathologic rarity. The tumor may respond favorably to radiation therapy, which should be employed only for surgically inaccessible tumors, and it may be curative.

Lymphangiosarcoma arising in chronic lymphedematous extremities

Data from 23 cases of lymphangiosarcoma associated with chronic lymphedema seen at the Mayo Clinic and 163 cases from the world literature are reviewed. The available information in 162 cases of postmastectomy lymphedematous lymphangiosarcoma revealed that the lesion occurred at an average age of 63.9 years (range 44 to 84 years) and an average of 10 years 3 months after mastectomy. Fifty per cent of the 129 patients eligible for follow‐up were dead within 19 months after treatment, and only 11 patients have survived 5 years or more. Amputation gave a slightly better prognosis than did radiation therapy. Of 24 patients (two from the Mayo Clinic) with lymphangiosarcoma and non‐postmastectomy lymphedema, 12 (50%) were dead within 34 months after treatment. Only two of the patients were long‐term survivors (5 and 15 years), and both were treated by amputation. Early recognition of the lesion and prompt radical ablative surgery seem to offer the best chance for survival.

Hemangioma, hemangiopericytoma, and hemangioendothelioma (angiosarcoma) of bone.

Sixty‐nine cases of bone tumors of vascular origin, excluding cases of lymphangiomas and massive osteolysis, were found in a complete review of the surgical files of the Mayo Clinic. There were 56 hemangiomas, and these often produced roentgenologic diagnostic problems. Most were easily managed surgically, although some vertebral hemangiomas required radiation therapy. There were known multicentric lesions in only 2 of the 56 cases. Hemangiopericytomas accounted for 4 of the 69 cases. In these 4, the clinical courses were characteristically unpredictable. There were 9 hemangioendotheliomas. These 9 were added to 13 cases in which specimens were submitted for review. In 6 of the 22 cases, there was multifocal involvement. In this group of 22 cases, it was impractical to differentiate a separate group and designate it as angiosarcoma. Half of the 22 patients died, 1 of known unrelated disease. Surgical extirpation, when possible, and irradiation of lesions incompletely excised or surgically inaccessible seem logical principles in treatment. In these cases of hemangioendotheliomas, the most important indicator of prognosis was the grade of anaplasia.

Chondromyxoid fibroma: A clinicopathologic study of 76 cases

A study of 76 chondromyxoid fibromas affirms that it is a benign tumor, apparently of cartilaginous derivation, that is most likely to occur in adolescents and young adults. Although a wide skeletal distribution has been observed, the lesion most often affects major limb bones, especially the tibia. The characteristic but somewhat complex histologic range of this lesion, coupled with its benign appearance on roentgenograms, allows ready distinction from other tumors, most importantly chondrosarcoma. The lesion is somewhat more aggressive and considerably more likely to recur in young patients, but adequate local resection nearly always can forestall such recurrence. Malignant transformation is extremely improbable.

Parosteal osteogenic sarcoma.

A clinicopathologic study of 79 patients with parosteal osteogenic sarcoma revealed that more females than males were affected and that most of the patients were in the second to the fourth decades of life. Sixty‐eight percent of the patients had involvement of the posterior aspect of the lower femoral shaft. The roentgenogram characteristically showed a large, dense lobulated mass attached by a broad base to the underlying bone but with no involvement of bone itself. Microscopically, the tumor presented as well‐formed bands of osteoid within a hypocellular spindle cell stroma. Seven of the lesions had foci of high‐grade osteogenic sarcoma within an otherwise typical parosteal osteogenic sarcoma. Six of the 79 lesions showed involvement of the medullary cavity at surgery. Thirty‐one patients had excision as their initial treatment; four of the 31 required no further therapy. Ten patients had resection initially; three of the ten had recurrence. Only four of 27 patients who underwent amputation initially developed pulmonary metastasis. The presence of histologically “active” tumor and medullary involvement seemed to affect the prognosis adversely. Our data indicate that complete radical removal of the the tumor is the treatment of choice, with resection when feasible and amputation when necessary.

Fibrosarcoma—a clinicopathologic and statistical study of 199 tumors of the soft tissues of the extremities and trunk

The records of all adult patients with soft‐tissue fibrosarcoma of the extremities or limb girdles treated at the Mayo Clinic between 1910 and 1968 were studied. Histologic sections of each tumor were reviewed. Of 330 patients with an original diagnosis of fibrosarcoma, 18 were excluded because of insufficient histologic material, and 113 were excluded because of revised diagnoses. The tumors from the remaining 199 patients were graded according to microscopic criteria. The 4 grades were correlated with information obtained at followup, and survival data were analyzed. A striking relationship of survival with grade was demonstrated. Patients selected for radical surgical treatment achieved improved survival for every category studied; however, there was no significant difference in survival for patients treated with wide excision as compared with those treated by amputation. Based on the more recent data from patients with primary fibrosarcoma, the probability of surviving 5 years is 60%.

Adjuvant radiation therapy for regional nodal metastases from malignant melanoma. A randomized, prospective study

After nodal metastasis from malignant melanoma, approximately 80% of patients die from disseminated disease. To clarify the role of radiation therapy (XRT) following node dissection, 56 patients with biopsy‐proven nodal metastasis participated in a randomized, prospective clinical trial which compares radiation therapy to the regional lymph node area following lymphadenectomy (27 patients) with lymphadenectomy alone (29 patients). Interesting differences in the survival curves (p = 0.09) and in the disease‐free interval curves (p = 0.08) for the two treatment groups proved to be attributable to imbalances in the age and nodal distributions in the treatment groups. Covariate analysis identified age and sex as the factors having the most significant (p < 0.04) effect on survival and identified the number of positive nodes as the covariate having the most significant (p < 0.02) effect on disease‐free interval. Treatment did not have a significant effect upon survival or disease‐free interval. Cancer 42:2206–2210, 1978.

Extra-articular synovial chondromatosis.

Four patients with extra-articular synovial chondromatosis and one patient with combined intra-articular and extra-articular involvement are described. In all five patients the lesion was anomalously located (knee, shoulder, hip, and ankle). The lesion usually occurs in the hands and feet. Accurate diagnosis is important, because if the synovial origin of the cartilaginous proliferations is ignored, the evidence of cellular activity may lead to an erroneous diagnosis of chondrosarcoma. All five patients had a benign clinical course.