Pathology and genetics of tumors of soft tissue and bone

Two patients underwent thoracotomy for resection of pulmonary or esophageal carcinoma. Postoperatively, epidural catheters were inserted for pain management. Complaints of severe injection pain over the abdomen or lower extremities were made during one administration of pain medication. Progressive weakness and numbness developed over the lower trunk and lower extremities, with subsequent respiratory difficulties. Potassium chloride (KCl) was suspected to have been mistaken for normal saline as the diluent for morphine. In addition to endotracheal intubation and ventilatory support, steroids were administered both intravenously and epidurally to suppress spinal cord irritation. The two patients regained motor and sensory functions 14 and 18 hours later, respectively, without sequelae.

Report of two cases

New uniform response criteria are required to adequately assess clinical outcomes in myeloma. The European Group for Blood and Bone Marrow Transplant/International Bone Marrow Transplant Registry criteria have been expanded, clarified and updated to provide a new comprehensive evaluation system. Categories for stringent complete response and very good partial response are added. The serum free light-chain assay is included to allow evaluation of patients with oligo-secretory disease. Inconsistencies in prior criteria are clarified making confirmation of response and disease progression easier to perform. Emphasis is placed upon time to event and duration of response as critical end points. The requirements necessary to use overall survival duration as the ultimate end point are discussed. It is anticipated that the International Response Criteria for multiple myeloma will be widely used in future clinical trials of myeloma.

International uniform response criteria for multiple myeloma

Historically, an adequate surgical procedure has been the most effective means of treating the majority of primary musculoskeletal sarcomas, and amputation has figured prominently in the surgical armamentarium. 4, 7, 9, 19, 21, 29, 41 The recent evidence that certain chemotherapeutic agents may have significant anti-sarcoma activity 2, 15, 17, 38 and coincident technical advances in irradiation therapy, radiographic localization, and reconstructive surgery have fostered enthusiastic interest in extremity-saving treatments. Almost all such treatments emphasize limb salvage as an alternative to amputation and are usually performed under a protective cloak of adjunctive chemotherapy, irradiation or immunoactive agents. 20, 23, 24, 30, 37, 39 Since neither chemotherapy nor irradiation therapy alone has been shown to assure long-term local control of bulk disease, surgical intervention remains an essential step in the overall management of musculoskeletal sarcomas. 3, 9, 17, 18, 29 Questions concerning the magnitude and timing of the surgical procedure are as unanswered as those relating to the most appropriate use of the adjuncts themselves. Increasingly, the surgeon and his patient are confronted with a bewildering array of therapeutic options, the long-term outcomes of which are unknown. These relatively rare sarcomas increasingly are distributed among a variety of treatment protocols in which multiple parameters differ. This trend necessitates interinstitutional cooperation if sufficient numbers of patients are to be available for the timely evaluation of treatments in clinical use. Such cooperation and even effective interinstitutional communication are seriously hampered by the lack of uniform language, so that meaningful comparison of treatments is currently impossible. Prime factors include the lack of a consistent definition of the surgery performed and a serviceable surgical staging system encompassing bone and soft tissue. Standard terminology will assure that like and unlike treatments are appropriately compared. Although an effective staging system should serve all members of the multidisciplinary team, the biologic behavior of musculoskeletal sarcomas suggests that the most useful staging system will articulate with the surgical procedure.

A system for the surgical staging of musculoskeletal sarcoma.

Objective To determine the clinical and laboratory features of newly diagnosed multiple myeloma. Patients and Methods Records of all patients in whom multiple myeloma was initially diagnosed at the Mayo Clinic in Rochester, Minn, from January 1, 1985, to December 31, 1998, were reviewed. Results Of the 1027 study patients, 2% were younger than 40 years, and 38% were 70 years or older. The median age was 66 years. Anemia was present initially in 73% of patients, hypercalcemia (calcium level ≥11 mg/dL) in 13%, and a serum creatinine level of 2 mg/dL or more in 19%. The β2-microglobulin level was increased in 75%. Serum protein electrophoresis revealed a localized band in 82% of patients, and immunoelectrophoresis or immunofixation showed a monoclonal protein in 93%. A monoclonal light chain was found in the urine in 78%. Nonsecretory myeloma was recognized in 3% of patients, whereas light-chain myeloma was present in 20%. Conventional radiographs showed an abnormality in 79%. The plasma cell labeling index was 1% or more in 34% of patients. Multivariate analysis revealed that age, plasma cell labeling index, low platelet count, serum albumin value, and the log of the creatinine value were the most important prognostic factors. Conclusion The median duration of survival was 33 months and did not improve from 1985 through 1998.

Review of 1027 Patients With Newly Diagnosed Multiple Myeloma

Aneurysmal bone cyst (ABC) is a nonneoplastic expansile bone lesion that mainly affects children and young adults. Primary ABC is relatively rare, with an incidence one half that of giant cell tumor of bone. In 238 patients with ABC studied in the Mayo Clinic files, more than 80% of the lesions were in long bones, flat bones, or the spinal column. Of the lesions initially treated at the Mayo Clinic, 95% were typical ABC; the rest were "solid" variants. Except for the absence of obvious cavernous channels and spaces, there was no significant histologic difference between solid variant and typical ABC. Radiographically, ABC is an eccentric expansile lesion commonly located at the metaphysis of long bones. Computed tomography and magnetic resonance imaging may show multiple internal septations or fluid levels. In the 153 patients treated, 19% had recurrence after curettage (intralesional excision). Recurrence was most common during the first 2 postoperative years.

https://onlinelibrary.wiley.com/doi/pdf/10.1002/1097-0142%2819920615%2969%3A12%3C2921%3A%3AAID-CNCR2820691210%3E3.0.CO%3B2-E

Aneurysmal bone cyst. A clinicopathologic study of 238 cases.

Our experience involving 221 consecutive patients with giant-cell tumor who were treated from 1960 to 1982 is reported. Of one group of 146 patients, twenty-seven who were initially treated by wide resection and 112 who had thorough curettage had a recurrence rate of 23 per cent after an average length of follow-up of seven years. All thirty-three recurrences were noted less than six years after operation, and twenty-seven were noted within the first three years postoperatively. Over-all, the type of surgical removal was the most significant factor in recurrence. The recurrence rate was 34 per cent in the patients who had curettage of the lesion and 7 per cent in those who had a wide resection. In a second group of seventy-five patients, initially treated by us for a recurrent tumor, there were fifteen subsequent recurrences, after an average length of follow-up of seven years. Curettage and bone-grafting, with preservation of function of the joint, is the preferred treatment for most patients.

Giant-cell tumor of bone

Osteoblastoma: Clinicopathologic study of 306 cases

BACKGROUND

Chondrosarcomas are common solid malignant tumors of bone, second in incidence only to osteosarcomas. The biologic evolution of chondrosarcomas is slow, requiring long follow-up intervals for meaningful survival analysis.



METHODS

This study describes the clinicopathologic profiles of 344 patients, 194 male and 150 female (M:F, 1.3:1.0), with primary chondrosarcoma of long bones and limb girdles seen at 1 institution over a period of 80 years.



RESULTS

The average age at presentation was 46 years (range, 5-82 years). The pelvis was the most common location (1.7% of all patients). Local pain was the most frequently reported initial symptom (81.4%). Survival analysis was limited to 233 patients whose primary treatment was given at the Mayo Clinic. All 233 patients had potential follow-up of at least 5 years. The overall 5-year survival rate was 77% (the expected rate was 96%). Local recurrence developed in 19.7% of patients and metastatic lesions in 13.7%. The recurrence rate was higher for tumors of the shoulder and pelvis than for tumors of long bones. Radiographically, chondrosarcomas had a characteristic appearance, including a combination of bone expansion and cortical thickening. Entering the tumor at surgery increased the risk of local recurrence. Histologic tumor grade was an important predictor of local recurrence and metastasis.



CONCLUSIONS

With adequate initial surgical intervention, chondrosarcoma is primarily a local disease with a low metastatic rate. Cancer 1998;83:2105-2119. © 1998 American Cancer Society.

https://acsjournals.onlinelibrary.wiley.com/doi/epdf/10.1002/%28SICI%291097-0142%2819981115%2983%3A10%3C2105%3A%3AAID-CNCR9%3E3.0.CO%3B2-U

Primary chondrosarcoma of long bones and limb girdles

Benign osteoblastoma is a rare tumor, more frequent in males, usually occurring in patients under the age of 30 years, and is seen most frequently in the spine, femur, tibia, and mandible. Its varied roentgenographic appearance may suggest a large osteoid osteoma or an aneurysmal bone cyst, but about one-fourth of cases present a picture consistent with a malignant neoplasm. The roentgen changes in the spine are frequently subtle and require care for identification. Differentiation from osteogenic sarcoma is sometimes difficult even with histological material, because some low grade osteogenic sarcomas contain areas resembling osteoblastoma. Conservative surgery is the treatment of choice for osteoblastoma.

Richard A. McLeod

Papers

Aneurysmal bone cyst. A clinicopathologic study of 238 cases.

Giant-cell tumor of bone

Osteoblastoma: Clinicopathologic study of 306 cases

Primary chondrosarcoma of long bones and limb girdles

The spectrum of osteoblastoma