- Email: [email protected]
Test and teach Number Twelve: Part 2
XIPELL &
ALLEN:
TEST AND TEACH
Pathology (1977), 9, pp. 268-9
TEST AND TEACH Number Twelve
Part 2 See p. 206 for details and photomicrographs of this case.
DIAGNOSIS: EWING’S SARCOMA, LEFT TIBIA According to Pomeroy & Johnson (1 9 7 9 , ‘Ewing’s sarcoma is morphologically characterized by uniform sheets of cells or cell nests outlined by fibrous stroma. The individual cells are round or oval with indistinct cytoplasmic borders, and are usually two or three times the size of lymphocytes. Nuclear margins are distinct and slightly irregular but not clefted, as may be observed with primary lymphomas of bone. Nucleoli are not prominent and mitoses are infrequent.’ Figures 1 & 2 illustrate these features. Pseudo-rosettes and a perithelial pattern, well recognized originally by Ewing (1922), are common in Ewing’s sarcoma, but are not illustrated here. Intracytoplasmic glycogen granules in tumour cells, which were found in this case, are usually seen in sections of Ewing’s sarcoma, particularly if alcoholic fixation or short-term exposure to 10% formalin fixative are employed, but in a minority of cases they are absent. Strong acid decalcifying agents such as nitric acid may dissolve the granules. Glycogen is not found in neuroblastomas or reticulum cell sarcomas of bone, the two tumours most commonly confused with Ewing’s sarcoma (Schajowicz, 1959). Examination for urinary catecholamines is helpful in distinguishing neuroblastomas from Ewing’s sarcomas, being positive in approximately 80% of neuroblastomas, including those in which rosette like structures are lacking. More recently it has been suggested that examination of the tumour tissue for monoamine oxidase may also be of assistance (Jeffree, 1974). Additional tumours occasionally misdiagnosed as Ewing’s sarcoma include other malignant lymphomas, primitive osteosarcoma, primary soft tissue round cell sarcoma (extra-skeletal Ewing’s sarcoma), adamantinoma, eosinophilic granuloma and histiocytosis X (Pomeroy & Johnson, 1975). Smears or imprints prepared from unfixed tissue at the time of biopsy may be helpful in finalizing a diagnosis, particularly when malignant lymphoma needs to be excluded. With the introduction of aggressive chemotherapy combined with radiotherapy, there has recently been an improvement in the prognosis of Ewing’s sarcoma of bone. In 1963, Bhansali & Desai reported an 8.5% 3-year survival rate in 82 of their patients, and an overall 8.7% average 5-year survival rate in all the large series reported to that time. In 1975, Pomeroy & Johnson reported a 2-year actuarial survival rate of 56% in a series of 66 consecutive patients treated at the National Cancer Institute by combined local irradiation of the primary site and various permutations of cyclophosphamide, vincristine, actinomycin D, methotrexate and whole brain irradiation. Factors associated with a worse prognosis were: a patient age of less than 10 years, initial metastatic disease, a high serum lactic dehydrogenase, systemic symptoms, leucocytosis, and a raised erythrocyte sedimentation
Pathology (1977). 9, July, p. 269
rate. Patients with a tumour in a limb bone had a better prognosis than those with a tumour in the trunk. The patient here illustrated was treated in air with local tourniquet anoxia and megavoltage radiotherapy to the tumour site in May and June 1974. This therapy was followed by a course of cyclophosphamide from July to September 1974. On 28 January 1976, 21 mth after diagnosis, she was well and had no evidence of recurrence.
ACKNOWLEDGEMENTS The manuscript was typed by S. Williamson. The Test and Teach series was initiated by the Board of Education of the Royal College of Pathologists of Australia. References BHANSALI, S. K. & DESAI,P. 8 . (1963): Ewing’s sarcoma. Observations on 107 cases. J . Bone Jt. Surg. 45A, 541-553. EWING,J. (1922): A review and classification of bone sarcoma. Arch. Surg. 4, 485-533. JEFFREE,G. M. (1974): Enzymes of round cell tumours of bone and soft tissue: a histochemical survey. J . Path. 113. 101-115.
POMEROY, T. C. &JOHNSON, R. E. (1975): Combined modality therapy of Ewing’s sarcoma. Cuncer (Philad.). 35, 3 6 4 7 . F. (1959): Ewing’s sarcoma and reSCHAJOWICZ, ticulum cell sarcoma of bone. J. Bone Jr. Surg. 41A, 349-356.
ALLEN:
TEST AND TEACH
Pathology (1977), 9, pp. 268-9
TEST AND TEACH Number Twelve
Part 2 See p. 206 for details and photomicrographs of this case.
DIAGNOSIS: EWING’S SARCOMA, LEFT TIBIA According to Pomeroy & Johnson (1 9 7 9 , ‘Ewing’s sarcoma is morphologically characterized by uniform sheets of cells or cell nests outlined by fibrous stroma. The individual cells are round or oval with indistinct cytoplasmic borders, and are usually two or three times the size of lymphocytes. Nuclear margins are distinct and slightly irregular but not clefted, as may be observed with primary lymphomas of bone. Nucleoli are not prominent and mitoses are infrequent.’ Figures 1 & 2 illustrate these features. Pseudo-rosettes and a perithelial pattern, well recognized originally by Ewing (1922), are common in Ewing’s sarcoma, but are not illustrated here. Intracytoplasmic glycogen granules in tumour cells, which were found in this case, are usually seen in sections of Ewing’s sarcoma, particularly if alcoholic fixation or short-term exposure to 10% formalin fixative are employed, but in a minority of cases they are absent. Strong acid decalcifying agents such as nitric acid may dissolve the granules. Glycogen is not found in neuroblastomas or reticulum cell sarcomas of bone, the two tumours most commonly confused with Ewing’s sarcoma (Schajowicz, 1959). Examination for urinary catecholamines is helpful in distinguishing neuroblastomas from Ewing’s sarcomas, being positive in approximately 80% of neuroblastomas, including those in which rosette like structures are lacking. More recently it has been suggested that examination of the tumour tissue for monoamine oxidase may also be of assistance (Jeffree, 1974). Additional tumours occasionally misdiagnosed as Ewing’s sarcoma include other malignant lymphomas, primitive osteosarcoma, primary soft tissue round cell sarcoma (extra-skeletal Ewing’s sarcoma), adamantinoma, eosinophilic granuloma and histiocytosis X (Pomeroy & Johnson, 1975). Smears or imprints prepared from unfixed tissue at the time of biopsy may be helpful in finalizing a diagnosis, particularly when malignant lymphoma needs to be excluded. With the introduction of aggressive chemotherapy combined with radiotherapy, there has recently been an improvement in the prognosis of Ewing’s sarcoma of bone. In 1963, Bhansali & Desai reported an 8.5% 3-year survival rate in 82 of their patients, and an overall 8.7% average 5-year survival rate in all the large series reported to that time. In 1975, Pomeroy & Johnson reported a 2-year actuarial survival rate of 56% in a series of 66 consecutive patients treated at the National Cancer Institute by combined local irradiation of the primary site and various permutations of cyclophosphamide, vincristine, actinomycin D, methotrexate and whole brain irradiation. Factors associated with a worse prognosis were: a patient age of less than 10 years, initial metastatic disease, a high serum lactic dehydrogenase, systemic symptoms, leucocytosis, and a raised erythrocyte sedimentation
Pathology (1977). 9, July, p. 269
rate. Patients with a tumour in a limb bone had a better prognosis than those with a tumour in the trunk. The patient here illustrated was treated in air with local tourniquet anoxia and megavoltage radiotherapy to the tumour site in May and June 1974. This therapy was followed by a course of cyclophosphamide from July to September 1974. On 28 January 1976, 21 mth after diagnosis, she was well and had no evidence of recurrence.
ACKNOWLEDGEMENTS The manuscript was typed by S. Williamson. The Test and Teach series was initiated by the Board of Education of the Royal College of Pathologists of Australia. References BHANSALI, S. K. & DESAI,P. 8 . (1963): Ewing’s sarcoma. Observations on 107 cases. J . Bone Jt. Surg. 45A, 541-553. EWING,J. (1922): A review and classification of bone sarcoma. Arch. Surg. 4, 485-533. JEFFREE,G. M. (1974): Enzymes of round cell tumours of bone and soft tissue: a histochemical survey. J . Path. 113. 101-115.
POMEROY, T. C. &JOHNSON, R. E. (1975): Combined modality therapy of Ewing’s sarcoma. Cuncer (Philad.). 35, 3 6 4 7 . F. (1959): Ewing’s sarcoma and reSCHAJOWICZ, ticulum cell sarcoma of bone. J. Bone Jr. Surg. 41A, 349-356.