25
Clinical Section
597
clinical illnesses, which showed quite different Recurrent Xanthogranuloma serological results. Initially rheumatoid arthritis (Malignant Fibrous Histiocytoma) was diagnosed on the basis of an erosive poly- H W S Pigott FRCS (for Harold Ellis Mch) arthritis in association with positive rheumatoid (Surgical Unit, Westminster Hospital, serology. There were no systemic features and London SW1) antinuclear antibodies were absent. This disease was satisfactorily controlled with reversal of the H C, woman aged 64. Housewife serological results and evidence of radiological History: Presented in February 1974 to another healing. The second illness was notable for its hospital with a history of right-sided abdominal lack of arthritic involvement and the presence of pain for two months. X-rays showed a retromultiple systemic manifestations. The DAT was peritoneal mass displacing the adjacent structures now negative and the latex test became negative and in March a perinephric retroperitoneal mass later. Antinuclear antibodies appeared in high (17 x 10 x 8 cm) was removed. May 1974: titre. There was an associated rise in DNA bind- referred to the Westminster Hospital with recuring capacity and lowering of serum complement, rent abdominal pain. supporting the diagnosis of systemic lupus ery- On examination: Large tumour palpable in right thematosus. Aspirin-induced hepatic impairment loin. IVP showed upward displacement of right also appeared with the onset of systemic disease, kidney. whereas previously the patient had tolerated Operation (28.5.74, H E): through a transverse regular aspirin. Aspirin-induced hepatotoxicity abdominal incision an 'en bloc' removal of the has previously been reported in association with tumour was performed including the adjacent systemic lupus erythematosus (Seaman et al. ascending colon, fundus of the gall-bladder, 1974). This patient would appear to demonstrate overlying abdominal wall, and part of the right the conversion from rheumatoid arthritis to lobe of the liver. The tumour measured 8 x 10 cm. On microscopic examination the appearances systemic lupus erythematosus rather than the were those of a malignant fibrous histiocytoma. coexistence of both diseases. Postoperatively the patient made an uneventful REFERENCES recovery and received a course of radiotherapy. Gumpel M, Ansell B M & Hall M A (1973) Excerpta Medica International Congress Series 299, 43 Kantor G L, Bickel Y B & Barnett E V (1969) American Journal ofMedicine 47, 433-444 Seaman W E, Ishak K G & Plotz P H (1974) Annals ofInternal Medicine 80, 1-8
Dr B M Ansell, in reply to questions, said that there was an immediate fall in polymorphs while the patient was taking cyclophosphamide, and a subsequent rise. Later, a reduction in polymorphs and lymphocytes was associated with thrombocytopenia without further cyclophosphamide. She did not know whether cyclophosphamide could be regarded as the cause of these changes. This was the only case known to ber to have
Discussion
Xanthogranuloma was the term used when the tumour was originally described (Oberling 1935). The cell of origin was demonstrated several years later to be the tissue histiocyte (Ozzello et al. 1963). Benign fibrohistiocytic lesions such as sclerosing angioma and fibrous histiocytoma are frequently found in the skin. Malignant tumours are also found, but the nomenclature is not entirely standardized. This case is an example of malignant fibrous histiocytoma. Reports of these rare tumours have suggested a high incidence of recurrence following local excision (Soule & Enriquez 1972). Occasional distant metastases have been recorded (Rosas-Uribe etal. 1970). Retroperitoneal histiocytomas are usually of the malignant fibrous type and are frequently found in the perirenal region. This case demonstrated the characteristic ebullient nodularity of the macroscopic specimen. Frequent foam cells, together with large histiocytes are seen on microscopic examination. Wide excisional surgery offers the best chance of cure in these rare tumours.
recovered from rheumatoid arthlitis and then developed systemic lupus erythematosus. Her experience was that a marked rise in transaminases on aspirin occurred only in Still's disease. Her patients-with rheumatoid arthritis had occasionally shown minor elevations, but liver disease had always been present. Dr T W Warnes (University College Hospital) said that elevated levels of serum phosphatase had been previously reported in patients with rheumatoid arthritis, and this finding had been thought to be unrelated to salicylate ingestion (Cockel R, Kendall M J, Becker J F & Hawkins C F, 1971, Annals of the Rheumatic Diseases 30, 166-170). Since 30% of patients with rheumatoid arthritis had elevated levels REFERENCES of serum 5-nucleotidase it would appear that sub- Oberling C (1935) American Journal of Cancer 23, 477-489 Ozzello L, Stout A P & Murray M R (1963) Cancer 16, 331-344 clinical hepatic dysfunction was common in rheuma- Rosas.Uribe A M & Rappaport H toid arthritis (Kendall M J, Farr M, Bold A M & (1970) CancerA,26.Ring 827-831 Soule E H & Enriqeez P (1972) Cancer 30, 128-143 Hawkins C F, 1971, Lancet ii, 1012-1013).
598 Proc. roy. Soc. Med. Volume 68 September 1975 Mr J S Kirkham (St James' Hospital, Balham) said he was surprised that the gall-bladder had been partially excised. He thought an ordinary cholecystectomy might have been both easier and safer; on the whole it caused little disability.
Dermatomyositis Associated with Carcinoma ofthe Breast P L Harris MD FRCS (for Ian Burn FRCS and J T Scott MD FRCP) (Charing Cross Hospital, Fulham Palace Road, London W6 8RF)
26 most commonly associated tumour (Williams 1959). Successful treatment of the tumour may result in remission of dermatomyositis (Mills 1971). Conversely persistence of dermatomyositis may indicate inadequate treatment of the tumour. In this patient oophorectomy failed to influence the progress of her occult malignant disease and the rash and myopathy persisted. Adrenalectomy caused regression of the tumour and the dermatomyositis subsided. The reason for this close relationship between dermatomyositis and malignant disease remains uncertain but an autoimmune process seems likely. Hypersensitivity to tumour extract has been reported in patients with dermatomyositis (Curtis et al. 1961) and more recently sensitization to muscle antigens of T lymphocytes from animals with experimentally induced myositis has been demonstrated (Manghani 1972). These results suggest a cell-mediated hypersensitivity reaction. The onset or exacerbation of malignant disease is often accompanied by a measurable immunological response (Field 1973). It may be that skin and muscle share with tumour tissue some common antigenic components toward which a cross-reacting antibody response is induced. As a result immune-injury occurs in skin and muscle as well as in tumour tissue.
J K, woman aged 48 History: Presented in September 1971 with painless lump in right breast which she first noticed four months previously. On examination: Mass 5 cm in diameter in upper outer quadrant of right breast. Enlarged but mobile nodes palpable in right axilla. Tests for distant metastases including liver function tests, chest and skeletal X-rays and bone and liver scans were negative. Biopsy confirmed diagnosis of carcinoma and she was treated by right radical mastectomy (Halsted). She remained free of symptoms until December 1973 when she complained of a rash on her face REFERENCES Curtis A C, Hechaman J H & Wheeler A H and upper arms, and pain and weakness in the (1961) Journal of the American Medical Association 178, 571-573 E J (1973) In: Modem Trend in Oncology. proximal muscles of the limbs. Erythematous Field Ed. R W Raven. Butterworths, London; pp 183-208 macular eruption over the malar area and cheeks Manghani D (1972) PhD Thesis, University of London and a heliotrope erythema of the upper eyelids Mills J A (1971) In: Dermatology in Internal Medicine. T B Fitzpatrick et al. McGraw-Hill, New York; pp 1518-1525 which was characteristic of dermatomyositis. Ed. Williams R C Muscle biopsy (quadriceps femoris) showed (1959) Annals ofInternal Medicine 50, 1117-1181 changes consistent with dermatomyositis. No sign of local or lymph-node recurrence of her carcinoma and no evidence of distant metastases on detailed investigation. The possi- Mr A J Edwards (Whipps Cross Hospital, Leytonstone) he was interested in Mr Harris's assertion that bility of occult metastases could not be excluded said dermatomyositic lesion was mediated by the and for this reason bilateral oophorectomy was the thymic-dependent pathway. There was considerable performed. Following this operation her rash evidence of enhancement of the collaborative thymicand myopathy increased in severity. Serum dependent lymphocytes in early animal tumours but creatine kinase rose from normal levels (
Clinical Section
597
clinical illnesses, which showed quite different Recurrent Xanthogranuloma serological results. Initially rheumatoid arthritis (Malignant Fibrous Histiocytoma) was diagnosed on the basis of an erosive poly- H W S Pigott FRCS (for Harold Ellis Mch) arthritis in association with positive rheumatoid (Surgical Unit, Westminster Hospital, serology. There were no systemic features and London SW1) antinuclear antibodies were absent. This disease was satisfactorily controlled with reversal of the H C, woman aged 64. Housewife serological results and evidence of radiological History: Presented in February 1974 to another healing. The second illness was notable for its hospital with a history of right-sided abdominal lack of arthritic involvement and the presence of pain for two months. X-rays showed a retromultiple systemic manifestations. The DAT was peritoneal mass displacing the adjacent structures now negative and the latex test became negative and in March a perinephric retroperitoneal mass later. Antinuclear antibodies appeared in high (17 x 10 x 8 cm) was removed. May 1974: titre. There was an associated rise in DNA bind- referred to the Westminster Hospital with recuring capacity and lowering of serum complement, rent abdominal pain. supporting the diagnosis of systemic lupus ery- On examination: Large tumour palpable in right thematosus. Aspirin-induced hepatic impairment loin. IVP showed upward displacement of right also appeared with the onset of systemic disease, kidney. whereas previously the patient had tolerated Operation (28.5.74, H E): through a transverse regular aspirin. Aspirin-induced hepatotoxicity abdominal incision an 'en bloc' removal of the has previously been reported in association with tumour was performed including the adjacent systemic lupus erythematosus (Seaman et al. ascending colon, fundus of the gall-bladder, 1974). This patient would appear to demonstrate overlying abdominal wall, and part of the right the conversion from rheumatoid arthritis to lobe of the liver. The tumour measured 8 x 10 cm. On microscopic examination the appearances systemic lupus erythematosus rather than the were those of a malignant fibrous histiocytoma. coexistence of both diseases. Postoperatively the patient made an uneventful REFERENCES recovery and received a course of radiotherapy. Gumpel M, Ansell B M & Hall M A (1973) Excerpta Medica International Congress Series 299, 43 Kantor G L, Bickel Y B & Barnett E V (1969) American Journal ofMedicine 47, 433-444 Seaman W E, Ishak K G & Plotz P H (1974) Annals ofInternal Medicine 80, 1-8
Dr B M Ansell, in reply to questions, said that there was an immediate fall in polymorphs while the patient was taking cyclophosphamide, and a subsequent rise. Later, a reduction in polymorphs and lymphocytes was associated with thrombocytopenia without further cyclophosphamide. She did not know whether cyclophosphamide could be regarded as the cause of these changes. This was the only case known to ber to have
Discussion
Xanthogranuloma was the term used when the tumour was originally described (Oberling 1935). The cell of origin was demonstrated several years later to be the tissue histiocyte (Ozzello et al. 1963). Benign fibrohistiocytic lesions such as sclerosing angioma and fibrous histiocytoma are frequently found in the skin. Malignant tumours are also found, but the nomenclature is not entirely standardized. This case is an example of malignant fibrous histiocytoma. Reports of these rare tumours have suggested a high incidence of recurrence following local excision (Soule & Enriquez 1972). Occasional distant metastases have been recorded (Rosas-Uribe etal. 1970). Retroperitoneal histiocytomas are usually of the malignant fibrous type and are frequently found in the perirenal region. This case demonstrated the characteristic ebullient nodularity of the macroscopic specimen. Frequent foam cells, together with large histiocytes are seen on microscopic examination. Wide excisional surgery offers the best chance of cure in these rare tumours.
recovered from rheumatoid arthlitis and then developed systemic lupus erythematosus. Her experience was that a marked rise in transaminases on aspirin occurred only in Still's disease. Her patients-with rheumatoid arthritis had occasionally shown minor elevations, but liver disease had always been present. Dr T W Warnes (University College Hospital) said that elevated levels of serum phosphatase had been previously reported in patients with rheumatoid arthritis, and this finding had been thought to be unrelated to salicylate ingestion (Cockel R, Kendall M J, Becker J F & Hawkins C F, 1971, Annals of the Rheumatic Diseases 30, 166-170). Since 30% of patients with rheumatoid arthritis had elevated levels REFERENCES of serum 5-nucleotidase it would appear that sub- Oberling C (1935) American Journal of Cancer 23, 477-489 Ozzello L, Stout A P & Murray M R (1963) Cancer 16, 331-344 clinical hepatic dysfunction was common in rheuma- Rosas.Uribe A M & Rappaport H toid arthritis (Kendall M J, Farr M, Bold A M & (1970) CancerA,26.Ring 827-831 Soule E H & Enriqeez P (1972) Cancer 30, 128-143 Hawkins C F, 1971, Lancet ii, 1012-1013).
598 Proc. roy. Soc. Med. Volume 68 September 1975 Mr J S Kirkham (St James' Hospital, Balham) said he was surprised that the gall-bladder had been partially excised. He thought an ordinary cholecystectomy might have been both easier and safer; on the whole it caused little disability.
Dermatomyositis Associated with Carcinoma ofthe Breast P L Harris MD FRCS (for Ian Burn FRCS and J T Scott MD FRCP) (Charing Cross Hospital, Fulham Palace Road, London W6 8RF)
26 most commonly associated tumour (Williams 1959). Successful treatment of the tumour may result in remission of dermatomyositis (Mills 1971). Conversely persistence of dermatomyositis may indicate inadequate treatment of the tumour. In this patient oophorectomy failed to influence the progress of her occult malignant disease and the rash and myopathy persisted. Adrenalectomy caused regression of the tumour and the dermatomyositis subsided. The reason for this close relationship between dermatomyositis and malignant disease remains uncertain but an autoimmune process seems likely. Hypersensitivity to tumour extract has been reported in patients with dermatomyositis (Curtis et al. 1961) and more recently sensitization to muscle antigens of T lymphocytes from animals with experimentally induced myositis has been demonstrated (Manghani 1972). These results suggest a cell-mediated hypersensitivity reaction. The onset or exacerbation of malignant disease is often accompanied by a measurable immunological response (Field 1973). It may be that skin and muscle share with tumour tissue some common antigenic components toward which a cross-reacting antibody response is induced. As a result immune-injury occurs in skin and muscle as well as in tumour tissue.
J K, woman aged 48 History: Presented in September 1971 with painless lump in right breast which she first noticed four months previously. On examination: Mass 5 cm in diameter in upper outer quadrant of right breast. Enlarged but mobile nodes palpable in right axilla. Tests for distant metastases including liver function tests, chest and skeletal X-rays and bone and liver scans were negative. Biopsy confirmed diagnosis of carcinoma and she was treated by right radical mastectomy (Halsted). She remained free of symptoms until December 1973 when she complained of a rash on her face REFERENCES Curtis A C, Hechaman J H & Wheeler A H and upper arms, and pain and weakness in the (1961) Journal of the American Medical Association 178, 571-573 E J (1973) In: Modem Trend in Oncology. proximal muscles of the limbs. Erythematous Field Ed. R W Raven. Butterworths, London; pp 183-208 macular eruption over the malar area and cheeks Manghani D (1972) PhD Thesis, University of London and a heliotrope erythema of the upper eyelids Mills J A (1971) In: Dermatology in Internal Medicine. T B Fitzpatrick et al. McGraw-Hill, New York; pp 1518-1525 which was characteristic of dermatomyositis. Ed. Williams R C Muscle biopsy (quadriceps femoris) showed (1959) Annals ofInternal Medicine 50, 1117-1181 changes consistent with dermatomyositis. No sign of local or lymph-node recurrence of her carcinoma and no evidence of distant metastases on detailed investigation. The possi- Mr A J Edwards (Whipps Cross Hospital, Leytonstone) he was interested in Mr Harris's assertion that bility of occult metastases could not be excluded said dermatomyositic lesion was mediated by the and for this reason bilateral oophorectomy was the thymic-dependent pathway. There was considerable performed. Following this operation her rash evidence of enhancement of the collaborative thymicand myopathy increased in severity. Serum dependent lymphocytes in early animal tumours but creatine kinase rose from normal levels (
