Intra-osseous synovial sarcoma is very rare and its mode of origin is conjectural. Our patient's tumour was situated within the lower femur. The sited of origin of the few intra-osseous synovial sarcomas so far documented may support the concept of intra-osseous synovial rests

Brawn, E

Rose, A G

Uys, C J

English

Cape Town University OpenUCT

SA MEDIESE TYDSKRIF 1 MEI 1982 673Intra-osseous synovial sarcomaA. G. ROSE, C. J. UYS, E. BRAWNSummaryIntra-osseous synovial sarcoma is very rare and itsmode of origin is conjectural. Our patient's tumourwas situated within the lower femur. The sites oforigin of the few intra-osseous synovial sarcomasso far documented may- support the concept ofintra-osseous synovial rests.'"S. AIr. med. J.. 61,673 (1982}.Tenosynovial sarcomas may take a variety of morphologicalforms and may be found at a variety of sites, the commonestbeing the thigh, foot, knee, shoulder and forearm. In all thesecommon locations and even in other rare sites, e.g. neck,abdomen and back, the tumour arises within soft tissues.We have recently encountered a primary synovial sarcoma'which arose de nrruo within the femur. Because of the extremerarity of such an occurrence and the possible support which itprovides for the concept of 'synovial rests' within long bones, thefollowing case is reported.Case reportA 16-year-old girl was admitted to hospital with a fracture of thelower end of the right femur resulting from a fall while walking.Radiography (Fig. I) showed that the fracture was due to amalignant tumour which appeared to be arising from within thebone. Biopsy of the lesion confirmed the presence ofa malignanttumour (Fig. 2), biphasic in character and composed of plump,spindle-shaped cells showing abundant mitotic activity, whileother areas showed narrow spaces lined by pseudo-epithelialcells. On histological examination the appearance was typical ofthe biphasic synovial sarcoma. Scattered osteoclast-like giantcells were present in some areas. Tomography of the chestrevealed no evidence of metastatic spread. The patientsubsequently underwent subtrochanteric amputation.Examination of the amputated leg confirmed the intra-osseousorigin of the tumour. Postoperative chemotherapy was refused.DiscussionSynovial sarcoma may involve bone, either by direct spread or byhaematogenous metastasis. The origin of such a tumour fromwithin bone appears to be an exceptional event, which is notmentioned in modern texts; in a survey of the recent literature weDepartment of Pathology, Groot~ Schuur Hospital andUniversity of Cape TownA. G. ROSE, M.MED. (PATH.)C. J. UYS, M.D, D.CLlN.PATH., F.R.e. (PATH.}Department of Histopathology, South African Institute forMedical Research, Port ElizabethE. BRAWN M.B. CH.B.Date received: 24 August 1981.Fig. 1. Radiograph of right leg showing pathological fracture through anarea of radiolucency caused by an intra-osseous osteolytic malignanttumour. Subperiosteal formation of new bone is observed proximal to thefracture.Fig. 2. Microscopic appearance of the tumour showing the formationwithin the sarcoma of spaces lined by pseudo-epithelial cells (H and E x150).have been able to find only a few reports. The tumour reportedby Srivastava and Gupta 1 in 1974 also involved the lower femur;synovial sarcomas arising within the tibia have been reported by674 SA MEDICAL JOURNAL 1 MAY 1982others~·3 and Evans el at. 4 reported a patient with a congenitalsynovial sarcoma of the humerus. A synovial origin has also beenmooted for adamantinoma of the tibia.The mode of origin of ynoviaI sarcoma from within bone iconjectural. Srivasrava and Gupta I proposed that fibroblasts andother mesenchymal cells within bone may be stimulated todifferentiate into synovial tissue which may undergo malignantchange. In a discussion regarding the pathogenesis of simplebone cysts, Mirra' suggested that they represent a congenital restof ynovial tissue which ha been displaced into the thin corticalmetaphyseal region of bones at the synovial-capsular bonereflection. The marked predominance of cysts at thehumerofemoral location can be explained by the fact that thesetwo bones have the largest area of capsular-to-metaphyseal bonereflection. It is not inconceivable that synovial sarcoma may alsoarise from such intra-osseous synovial rests.The presence of giant cells within the stroma of the tumour inour patient is probably related to the intra-osseous situation ofthe tumour; similar giant cells were also present in the synovialsarcoma of the tibia reported by Lederer and Sinclair. 3'REFERE:--:CESI. Srivasrava, T. P. and Gupra, S. (1974): 1nl. Surg., 59, 303.2. Hicks, J. D. (195-1): J. parh. Bacl., 67,151.3. Lederer, H. and Sinclair, .-\. J. (1954): Ibid., 67, 163.4. Evans, R. W., Thomas, G. E. and Walker, N. M. (1960): J. Bone Jl. Surg.,42B,742.5..\1irra, J. .\1. (1980): Bone Tumours: DitiglllJ:H·~· and TrealJJlt.'w, p. 468.Philadelphia: J. E. Lippincotl.Severe airway obstructionwith rheumatoid arthritissyndromeA case reportassociatedand Sjogren'sM. B. FORMAN, S. ZWI, A. J. GEAR, J. KALLENBACH, J. WINGSummaryNumerous pulmonary complications associatedwith rheumatoid arthritis (RA) and Sj6gren'ssyndrome (SS) have been described. Mild airwayObstruction has recently been recognized in thesedisorders, severe obstruction occurring in only afew patients with RA. We report a patient with RAand SS who developed severe irreversible airwayobstruction, an association not hitherto described.The airway disease failed to respond tobronchodilators, steroids or immunosuppressiveagents.S. AIr. med. J.. 61, 674 (1982).Numerous pulmonary manifestation of both rheumatoidanhritis \~) and Sj6gren's syndrome (SS) have beendescribed. -~ Although airway obstruction in RA has been notedfor some time, this complication was o~~~inally attributed to thesmokmg habIts of the panents studied. Later studies revealedRheumatology and Pulmonology Units, JohannesburgHospital and University of the Witwatersrand,JohannesburgM. B. FORMAN, .\I.B. B.CH.•.\I.R.C.P.S. ZWI, B.SC, M.B. B.CH., F.C.CP.• F.R.CP.A. J. GEAR, .\1.B. B.CH., F.c.P. (S.A.)J. KALLENBACH, .\I.B. B.CH.•.\I.R.CP.l- WING, .\I.B. B.CH.Date received: 9 July 1981.Reprint requests [0: Professor S. Zwi. Depr of !\ledicine, .\1edical School, L:niversin' of theWitw3tersrand, Hospital Street, Johannesburg, 2001 RSA, .mild airway obstruction in a significant proportion of patientswith RA who were non-smokers. 4.9 Recently a severe form ofairway obstruction, characterized by obliterative bronchiolitis,has been described in a few patients with RA.2 In contrast, SS isusually associated with restrictive ventilatory defects, 3 andobstructive airway disease has been reported only in I study. IQWe report a patient with seropositive RA and SS associated withsevere irreversible airway obstruction. The lung disease hasremained refractory to treatment with bronchodilators, steroidsand immunosuppressive agents.Case reportA 54-year-old White woman presented in October 1979 withsymmetrical polyarthritis involving the feet, wrists, proximalinterphalangeal joints, shoulders, elbows and knees. Arheumatoid factor test was positive, and she was treated withseveral non-steroidal anti-inflammatory drugs with poorsymptomatic relief. She was referred to the Rheumatology Clinicat the Johannesburg Hospital 1 year later for further assessment.In addition to joint pain she complained of severe grittiness ofthe eyes and a dry mouth for the previous few months. Althoughher physical activity had been severely curtailed by the arthritis,a progressive decrease in effort tolerance and a non-productivecough had been present for the past few months. There was nopast history of cough, asthma, or exposure to. industrial orchemical agents. She was a non-smoker, had never been treatedwith steroids, penicillamine or gold, and denied any antecedentchest infections.Examination revealed typical rheumatoid changes in thehands and feet with acute synovitis of the wrists, proximalinterphalangeal joints, metacarpophalangeal joims, shoulders,knees, ankles and toes. She was tachypnoeic at rest andgeneralized fine inspiratory crepitations were audible, mostmarked at the midzones and bases of both lungs. The rest of theexamination was negative. Investigation showed a mildhypochromic microcytic anaemia (haemoglobin concentration

Intra-osseous synovial sarcoma.

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Intra-osseous synovial sarcoma.

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