The pathology of malignant fibrous histiocytoma of bone. A study of 130 patients.

Since 1927, 130 patients with well-documented malignant fibrous histiocytoma of bone have been diagnosed and treated at Memorial Hospital for Cancer and Allied Diseases. This sarcoma is 10 times less frequent than osteogenic sarcoma in this hospital. It most commonly occurred spontaneously (72%), whereas in the rest (28%) it followed previous radiation or various pre-existent osseous conditions, most often Paget's disease. The appendicular skeleton was the commonest site of involvement. The majority of the patients were middle-aged or older adults with a mean of 40.5 years of age; only 21.5% were 21 years or younger. Histologically, the lesions were subclassified as fibrous (62%), histiocytic or xanthomatous (30%), and malignant giant cell tumor (8%) variants. Older patients were more likely to have a secondary malignant fibrous histiocytoma, especially following radiation or Paget's disease. Overall survival estimates at 2 years and 5 years were 71% and 53%, respectively. Survival was not dependent on the histologic subtype of the lesion, but was strongly influenced by the histologic grade of malignancy. Important prognostic factors were the age of the patients and whether the lesions were primary de novo or secondary sarcomas: the older patients and those with secondary lesions did substantially worse.

Osteogenic sarcoma in pregnant women. Prognosis, therapeutic implications, and literature review.

This study of 18 pregnant women with concomitant osteogenic sarcoma of bone analyzes the important assertion whether this sarcoma and pregnancy have an adverse interaction. For comparison we matched the pregnant osteogenic sarcoma patients with nonpregnant women with the same skeletal tumor location and histologic appearance as well as similar age distribution. There was no worsening of prognosis of pregnant osteogenic sarcoma patients, and neither the pregnancy nor the disease appeared to act adversely toward the other. The 18 pregnant women with osteogenic sarcoma fared no better (nor worse) than the nonpregnant women with osteogenic sarcoma.

Osteogenic sarcoma of the skull. A clinicopathologic study of 19 patients.

The authors studied 19 patients with well documented osteogenic sarcomas arising in the skull, which represent 1.6% of all osteogenic sarcomas registered during a 60-year period (1921-1981). Ten sarcomas were primary, de novo tumors. Nine others developed secondary osteogenic sarcomas; among these, six arose as a complication of Paget's disease, two followed irradiation, and one was associated with pre-existent fibrous dysplasia. The sarcomas arose in equal proportion in both sexes with the men being much older (mean age, 44 years) as compared to the women (mean age, 31 years). Patients with de novo osteogenic sarcomas were considerably younger than those with secondary lesions. Osteoblastic osteogenic sarcoma was by far the most common histologic variant in both the primary and the Paget's sarcomas. None of the patients with Paget's sarcoma lived longer than 1 year; the median survival here was 4 months. Patients with de novo osteogenic sarcomas fared much better and there are four long-term survivors (longer than 3 years) who are currently disease-free.

Postradiation osteogenic sarcoma of bone and soft tissues. A clinicopathologic study of 66 patients.

Sixty-six patients with well-documented osteogenic sarcomas arising in bones and soft tissues after exposure to x-rays, which represent approximately 5.5 percent of all osteogenic sarcomas registered since 1921 at this institution, were studied. These secondary sarcomas occurred in equal proportion in both sexes, with the sixth decade of life being the most common age. In 42 patients, the bone had been normal at the time of irradiation, whereas in 24, the radiation was directed against an osseous tumor or tumor-like lesion. The median latent period was 10.5 years in both groups, ranging from 3.5 to 33 years. The radiation varied from diagnostic quality to 1 MeV x-rays. The dose was variable, but none was less than 2000 rads. Postradiation osteogenic sarcomas most commonly arose in the bones of the pelvic and shoulder regions. Histologically, the sarcomas were mostly of the fibrous type (46%) and radiographically showed a destructive bone lesion with or without signs of radiation osteitis. The cumulative disease-free survival rate at 5 years was 17%, with a median survival estimate of 1 year.

Age and prognosis in neuroblastoma. Review of 112 patients younger than 2 years.

The results of 112 children with neuroblastoma treated at the Memorial Sloan-Kettering Cancer Center between 1949 and 1980 were analyzed. Of these children, 58 were 0-11 months old and 54 were 12-23 months old and there was a median follow-up of 111 months. All 10 patients with Stage I are alive, 21/27 with Stages II and III (78%) are alive, 5/67 patients (7%) with Stage IV are alive, and 7/8 patients with Stage IVS are alive. Age of the children is an independent prognostic factor. The survival of infants with Stage IV is significantly better than it is for older children of the same stage. Two of 15 infants in Stages II and III died, both of early complications, whereas 4/12 older children with the same stages died. Minimal individualized treatment is recommended for children 0-11 months old who have localized and Stage IVS neuroblastoma. Children less than 1 year old with localized and Stage IVS neuroblastoma had an extremely good prognosis (90% survival) and were usually cured without intensive chemotherapy. Surgical removal of the primary tumor was sufficient for Stage I, and partial tumor removal followed by conservative radiation or chemotherapy was sufficient in most Stage II and III patients. Gentle, individualized treatment was adequate for Stage IVS. Children less than 1 with Stage IV neuroblastoma had a significantly better prognosis than older children of the same stage, but their prognosis was still poor (18% survival).

Osteogenic sarcoma in the American black.

This clinicopathologic study of 100 American black patients with osteogenic sarcoma diagnosed and treated at this Medical Center from 1921-1979, inclusive, demonstrates a progressively increasing proportion of black patients admitted for this disease. The relative upsurge became especially pronounced in the 1970s. The ages of the patients ranged from 3 to 58 years (median, 16, mean 19.2 years). The age distribution shows that blacks, on the whole, are younger than whites when they develop these tumors, and this is particularly evident in the black girls. Skeletal locations for osteogenic sarcomas, in general, are similar in both races, except that the tibia and the fibula were involved significantly more frequently in the blacks while the humerus was afflicted less commonly. The clinical stage of disease on presentation, the duration of signs and symptoms, the histologic subclassification of the tumors, and the radiographic appearances closely matched in both races. The numbers of patients with Paget's sarcomas were also evenly distributed. Twenty-nine patients are currently alive with the 2-year and 5-year disease-free survival being 42% and 32%, respectively. There are no differences in the survival of black as compared to white patients, either for the entire duration of the study or for the period after 1974. The poorer prognosis of cancer in blacks does not apply to osteogenic sarcoma patients.

Osteogenic sarcoma associated with Paget's disease of bone. A clinicopathologic study of 65 patients.

Among 1177 osteogenic sarcoma patients diagnosed and treated at Memorial Hospital, 65 (5.5%) were associated with either monostotic or polyostotic Paget's disease. The overall median age was 64 years (range, 39-82 years). In those patients older than 40 years of age, the frequency of sarcomatous transformation rose to 27%. There were slightly more men (55%) than women. The most common skeletal sites were the pelvic bones (34%), the humerus (22%), the femur (19%), and the craniofacial bones (14%). Unrelenting pain and tender swelling were the most common presenting symptoms (85%), with pathologic fracture in 14 (22%) patients. In two-thirds of the cases, the radiographic presentation was that of a lytic destructive lesion; while in the others it showed a sclerotic, mixed, or permeative character. In almost one-half of the cases, the histologic appearance of the osteogenic sarcomas was either fibrohistocytomatous or osteoblastic. In spite of radical surgical amputations, only three patients survived longer than 5 years. The prognosis of Paget's sarcoma is significantly less favorable than in osteogenic sarcoma arising de novo in patients of comparable age.

Primary lymphomas of the gastrointestinal tract. Analysis of prognostic factors with emphasis on histological type.

We have analyzed the clinicopathological factors affecting survival in 60 primary gastrointestinal lymphomas seen at Memorial Hospital between 1949 and 1978. Patients with generalized lymphoma (Stages III and IV) at the time of diagnosis and those without follow-up information or adequate histological material were excluded from this study. Lymphomas were classified according to the Lukes-Collins, Kiel, and Rappaport schemes and the patients were staged retrospectively by a modified Ann Arbor system. The patients were treated by surgical resection, radiotherapy, or both. Survival was influenced by histological type (P = 0.0116), stage of the disease (P less than 0.0001), and size of the primary tumor (P = 0.0007). Low-grade lymphoplasmacytoid lymphomas, recognized in 26.6% of the cases, had a low rate of extra-abdominal recurrence; 74% of these patients were alive without evidence of recurrence after a median follow-up of 171 months, or died without evidence of lymphoma with a median survival of 147 months. Centrocytic (Kiel) or cleaved cell (Lukes-Collins) types were seen in 13% and 21%, and high grade (Kiel) or large noncleaved and immunoblastic (Lukes-Collins) in 33.3% and 30% of the cases, respectively. These groups had a high rate of extra-abdominal recurrences, and over 60% of the patients died of lymphoma, with a median survival of 8 for the centroblastic-centrocytic and 7 months for the high-grade tumors. Histological type and clinicopathological staging emerge as useful factors for the identification of patients with high risk of systemic recurrence, probably best treated with chemotherapy in addition to surgery and local radiotherapy.

Telangiectatic osteogenic sarcoma: a clinicopathologic study of 124 patients.

One hundred-twenty-four patients with this rare and special variant of osteogenic sarcoma were treated at Memorial Sloan-Kettering Cancer Center from 1921 through 1979, representing 11% of all of osteogenic sarcomas. The lesions were predominantly lytic, destructive tumors with only minimal sclerosis on roentgenograms and soft as well as cystic on gross examination. Histologically, aneurysmally dilated spaces lined or traversed by sarcoma cells producing osteoid were noted. The differential diagnosis both radiographically and histologically included several benign lesions like aneurysmal bone cyst and giant cell tumor, among many others. It was found that telangiectatic osteogenic sarcoma is relatively frequent in the femoral diaphysis and in the distal end of the femur. Twenty-nine percent of the patients present with pathologic fracture, or this develops later. Age and sex distribution, or clinical signs or symptoms were those of ordinary osteogenic sarcomas. No differences in survival rates were found in lesions that were purely lytic or those with minimal sclerosis. Similarly, no differences in survival were noted when comparing patients with telangiectatic or ordinary osteogenic sarcoma. As a matter of fact, definite increase in survival was found in patients treated since 1975 with preoperative multidrug chemotherapy employing high-dose methotrexate. Adriamycin, and the combination of bleomycin, cyclophosphamide, and dactinomycin.