308
Journal of the Royal Society of Medicine Volume 84 May 1991
tumid erythematous nodules which progressed to pustulation and then ulcerated. Azathioprine (initial dose 25 mg daily, maximum dose 100 mg daily), methotrexate (initial dose 2.5 mg/week, maximum dose 7.5 mg/week) and potassium iodide (initial dose 50 mg daily, maxmum dose 500 mg three times daily) were tried in succession for 2 months, 2 months and 10 days respectively, but did not affect the steady progression of her cutaneous disease. Prednisolone (initial dose 30 mg daily, maximum dose 60mg daily) therapy was accompanied by the rapid onset of marked cushingoid changes, steroid myopathy and subsequently opportunistic bronchopneumonia. Thalidomide (dose 100 mg twice daily) was prescribed for 4 months but caused nausea and vomiting and failed to halt the progression of the panniculitis. Screening of our patient's serum identified a moderate level of circulating immune complexes. It was considered that these could be relevant to the development :nd maintenance of the panniculitis therefore plasmaphoresis was performed but appeared to make very little immediate difference. As part ofthe planned treatment, p phoresis was followed by cyclophosphamide infusios with an initial dose of 1250 mg followed by 750 mg at 3-monthly intervals. Our patient has now received two such infusions and there has been a partial remission in her cutaneous disease with a significant reduction in deep pustulation. Discussion Adipose tissue may be involved as part ofthe inflammatory process of connective tissue in lupus erythematosus, scleroderma and dermatomyositis'. There are few reports of panniculitis occurring in rheumatoid arthritis24 and we
are aware of no detailed case reports after Yaffee's case in 1955. Many of the drugs used unsuccessfully in the treatment of our patient have been reported to be effective in the treatment of panniculitis. A discussion of such treatments is beyond the scope of this report but can be obtained from current authoritative textbooks5. Clinical and histological features of our case are similar to those described in connective tissue panniculitis'. This seldom reported type of panniculitis follows a progressive course with chronic caseous necrosis of fat tissue, a lymphohistiocytic infiltrate and results in marked subcutaneous atrophy. Antimalarials have been reported to be effective in connective tissue panniculitis but previous intolerance precluded their use in our patient. References 1 Winkelmann RK. Panniculitis in connective tissue disease. Arch Dermatol 1983;119:336-44 2 Winkelmann RK, Peters MS. Lupus panniculitis. In: Moschella SL, ed. Dermatology update. New York: Elsevier 1982:135-52 3 Weber FP. A case of relapsing non-suppurative nodular panniculitis, showing phagocytosis of subcutaneous fat-cells by macrophages. Br J Dermatol 1925;37:301-11 4 Yaffee HS. A peculiar nodosity associated with arthritis. US Armed Forces Med J 1955;6:1043-52 5 Rook A, Wilkinson DS, Ebling FJG, Champion RH, Burton JL. Textbook of dermatology, 4th edn. Oxford: Blackwell Scientific, 1986:1870
(Accepted 3 January 1991. Correspondence to Dr A Anstey, Department of Dermatology, St Bartholomew's Hospital, West Smithfield, London EClA 7BE)
Malignant retroperitoneal schwannoma
Case presented to Clinical Section, 8 February 1991
N D Heaton FRCS A C Page FRCR E R Howard MS FRCS Firm III Office, Department of Surgery, King's College Hospital, Denmark Hill, London SE5 8RX Keywords: retroperitoneal tumours; malignant schwannoma
Primary retroperitoneal neurilemmomas are rare tumours in the absence of Von Reklinghausen's disease. Fewer than 50 benign and 20 malignant cases have been described in the English literature. A patientis described prentingwith vague upper abdominal symptoms who was found to have a 3 kg malignant retroperitoneal neurilemmoma associated with the pancreatic tail. Case report A 60-year-old man presented with a 6 week history of oesophageal regurgitation and post-prandial fullness. There had been no change in his bowel habit and his weight had been constant. On examination there was a large, firm nontender mass in the left upper quadrant that did not move with respiration. There were no cutaneous stigmata of neurofibromatosis. A barium meal revealed a huge mass filling the lesser sac and displacing the stomach to the left (Figure 1). An ultrasound scan showed it to consist of cystic and solid elements and it was thought to arise from the tail of the pancreas. A CT scan confirmed the multicystic appearance of the lesion and no liver or lymph node metastases were evident. Fine needle aspiration cytology and CT guided
Figure 1. Barium meal showing displacement ofthe les curvature of the stomach to the left by a retroperitoneal mass
needle biopsies were taken and showed features of a low grade soft tissue tumour, but no specific diagnosis was reached. A digital subtraction coeliac angiogram revealed marked displacement of the superior mesenteric, spleic and portal veins by a large avascular mass.
14IO7"'IV6 0503O8O2/$O2.00/0 o 1991 The Royal Society of Medicine
Journal of the Royal Society of Medicine Volume 84 May 1991
UFigure 2. CT scan of the multicystic mass arising from the tail of the pancreas
At operation the tumour extended from the base of the transverse mesocolon to the fundus of the stomach and appeared to arise from the body and tail ofthe pancreas. The tumour was adherent to, but not obviously infiltrating, the posterior wall of the stomach. There was no evidence of metastatic spread. The lesion was dissected free from the posterior peritoneum and removed with the neck, body and tail of the pancreas and the spleen. There were no postoperative complications. The tumour weighed 3.1 kg and showed the features of a malignant schwannoma. There were up to 3/10 mitoses per high powered field in the cellular areas ofthe tumour, which appeared to be well circumscribed and not invading the pancreas. Nine months after the operation he developed abdominal pain and was found to have multiple secondary deposits in the liver and retroperitoneum. Discussion Retroperitoneal malignant schwannomas in the absence of neurofibromatosis are rare, representing fewer than 2% of all primitive tumours occurring in the region'. Das Gupta2 found only four retroperitoneal tumours in 232 patients with solitary malignant schwannomas not associated with neurofibromatosis. Fewer than 20 cases have been described in detail in the English and 55 cases in the Japanese literature3. Patients typically present with vague upper abdominal pain and a palpable epigastric or left upper quadrant mass4'5. Infection and haemorrhage may complicate or precipitate presentation. Preoperative diagnosis is difficult even with ultrasound and CT scanning6. Seventy-five per cent of malignant retroperitoneal schwannomas have
Testicular lymphoma with pancreatitis
C Ade MB BS1 M Hatton MB BS' P Hamilton Stewart MS FRCS2 B Naylor FRCPath3 D Parker DPhil MRCP1 1Oncology Unit, 2Urology Unit, and 3Department of Histopathology, Bradford Royal Infirmary, Bradford BD9 6RJ Keywords: non-Hodgkin's lymphoma; testis; pancreatitis
A patient with testicular high-grade non-Hodgkin's lymphoma is described who developed obstructive jaundice and
309
cystic changes on imaging often leading to msdiagnosis of pancreatic pseudouy-. Asiratoytbiogy- or trucut biopsy seldom providle sufficent tissue for accurate diagnosis8. These tumours grow to a g s; 8 jitres of fluid was drained from one cystic lesion and narked tumour vascularity has been describedi paticula'in benign schwannomas8. Preoperative airs hy has been , operability and recommended to assess tumour v the origin of tumour blood supply6. The tumour in our g y provided patient was avascular and although a useful information concerning the vaclar ply it was of little diagnostic help. Surgical treatment offers the best prepecteofeur or longterm palliation9. Recurrence, liowever, his usually been reported within 6 mont of rtgical treatment10. The prognosis of malignant retrolpritoeal schwannoma is apparently worse if asoated with nqurofibromatosis2.11. Radiotherapy has been used preoperatively to reduce tumour vascularity, particularly in benign retroperitoneal schwannomas8, but the malignant form' appears to be unresponsive. The use of chemotherapy has been limited and the results inconclusive, however, adriamycin has been the most active drug tried4. The patient is alive at 15 months. References 1 Sordillo PP, Helson L, Hajdu SI, et aL Malignant schwannoma clinical characteristics, survival and response to therapy. Cancer 1981;47:2503-9 2 Das Gupta TK, Brasfield RD. Solitary malignant schwannoma. Ann Surg 1970;171419-28 3 Miyagi T, Shimamura M. Retroperitoneal schwannoma: a report of two cases and review of the literature. Hinoyokika-Kiyo 1986;32:207-14 4 Ball JHS, Sonnendecker EWW, Sevitz H, Murray J. Retroperitoneal malignant schwannoma. S Afr Med J 1987;71:49-52 5 McCarthy S, Duray PH. Giant retroperitoneal neurilemmoma: a rare cause of digestive tract symptoms. J Clin Gastroenterol 1983;5:343-7 6 Pandolfo I, Fiumara F, Chirico G, La Spada F, Caminiti R. Computed tomography in retroperitoneal neurofibrosarcoma (malignant schwannoma). J Comput Assist Tomogr 1983; 7:1119-20 7 Takatera H, Takaha M, Takiuchi H, Ohnishi S, Namiki M, Sonada T. Retrperitoneal schwannoma. Urology 1986;28:529-31 8 Regan JF, Juler GL, Schmutzer KJ. Retroperitoneal neurilemmoma. Am J Surg 1977;134:140-5 9 Moazam F, Rogers BM, Talbert JL. Retroperitoneal malignant schwannoma: a case report. J Pediatr Surg 1983;18:189-92 10 White HR. Survival in malignant schwannoma. Cancer
1971;27:720-9 11 Ghosh BC, Ghosh L, Huvos AG, Fortner JG. Malignant schwannoma. A clinicopathologic study. Cancer 1973;31:184-90
(Accepted 11 December 1990)
pancreatitis due to lymphomatous infiltration of the pancreas. He responded to cytotoxic chemotherapy and remains well one year after treatment.
Case report A 46-year-old man developed swelling and pain in the left testis over a period of 5 weeks. In July 1988, he had a left orchidectomy and histology showed high-grade non-Hodgkin's lymphoma of B cell origin. In November 1988, he developed an illness consisting ofback pain, anorexia and steatorrhoea. He was admitted to hospital with polyuria, polydipsia and glycosuria. On mination he was jaundiced and was tender in the left upper quandrant of the abdomen. Investigations showed blood glucose 13.2 mmol/l, serum amylase 908 IU/1, bilirubin 116 ,mol/l, alkaline phosphatase 418 IU/l and gamma glutamyl transpeptidase 675 IU/1. CT scan of the abdomen showed slight pancreatic enlargement (Figure 1)
0141-0768/91/ 050309-02/$02.00/0 © 1991 The Royal Society of Medicine
Journal of the Royal Society of Medicine Volume 84 May 1991
tumid erythematous nodules which progressed to pustulation and then ulcerated. Azathioprine (initial dose 25 mg daily, maximum dose 100 mg daily), methotrexate (initial dose 2.5 mg/week, maximum dose 7.5 mg/week) and potassium iodide (initial dose 50 mg daily, maxmum dose 500 mg three times daily) were tried in succession for 2 months, 2 months and 10 days respectively, but did not affect the steady progression of her cutaneous disease. Prednisolone (initial dose 30 mg daily, maximum dose 60mg daily) therapy was accompanied by the rapid onset of marked cushingoid changes, steroid myopathy and subsequently opportunistic bronchopneumonia. Thalidomide (dose 100 mg twice daily) was prescribed for 4 months but caused nausea and vomiting and failed to halt the progression of the panniculitis. Screening of our patient's serum identified a moderate level of circulating immune complexes. It was considered that these could be relevant to the development :nd maintenance of the panniculitis therefore plasmaphoresis was performed but appeared to make very little immediate difference. As part ofthe planned treatment, p phoresis was followed by cyclophosphamide infusios with an initial dose of 1250 mg followed by 750 mg at 3-monthly intervals. Our patient has now received two such infusions and there has been a partial remission in her cutaneous disease with a significant reduction in deep pustulation. Discussion Adipose tissue may be involved as part ofthe inflammatory process of connective tissue in lupus erythematosus, scleroderma and dermatomyositis'. There are few reports of panniculitis occurring in rheumatoid arthritis24 and we
are aware of no detailed case reports after Yaffee's case in 1955. Many of the drugs used unsuccessfully in the treatment of our patient have been reported to be effective in the treatment of panniculitis. A discussion of such treatments is beyond the scope of this report but can be obtained from current authoritative textbooks5. Clinical and histological features of our case are similar to those described in connective tissue panniculitis'. This seldom reported type of panniculitis follows a progressive course with chronic caseous necrosis of fat tissue, a lymphohistiocytic infiltrate and results in marked subcutaneous atrophy. Antimalarials have been reported to be effective in connective tissue panniculitis but previous intolerance precluded their use in our patient. References 1 Winkelmann RK. Panniculitis in connective tissue disease. Arch Dermatol 1983;119:336-44 2 Winkelmann RK, Peters MS. Lupus panniculitis. In: Moschella SL, ed. Dermatology update. New York: Elsevier 1982:135-52 3 Weber FP. A case of relapsing non-suppurative nodular panniculitis, showing phagocytosis of subcutaneous fat-cells by macrophages. Br J Dermatol 1925;37:301-11 4 Yaffee HS. A peculiar nodosity associated with arthritis. US Armed Forces Med J 1955;6:1043-52 5 Rook A, Wilkinson DS, Ebling FJG, Champion RH, Burton JL. Textbook of dermatology, 4th edn. Oxford: Blackwell Scientific, 1986:1870
(Accepted 3 January 1991. Correspondence to Dr A Anstey, Department of Dermatology, St Bartholomew's Hospital, West Smithfield, London EClA 7BE)
Malignant retroperitoneal schwannoma
Case presented to Clinical Section, 8 February 1991
N D Heaton FRCS A C Page FRCR E R Howard MS FRCS Firm III Office, Department of Surgery, King's College Hospital, Denmark Hill, London SE5 8RX Keywords: retroperitoneal tumours; malignant schwannoma
Primary retroperitoneal neurilemmomas are rare tumours in the absence of Von Reklinghausen's disease. Fewer than 50 benign and 20 malignant cases have been described in the English literature. A patientis described prentingwith vague upper abdominal symptoms who was found to have a 3 kg malignant retroperitoneal neurilemmoma associated with the pancreatic tail. Case report A 60-year-old man presented with a 6 week history of oesophageal regurgitation and post-prandial fullness. There had been no change in his bowel habit and his weight had been constant. On examination there was a large, firm nontender mass in the left upper quadrant that did not move with respiration. There were no cutaneous stigmata of neurofibromatosis. A barium meal revealed a huge mass filling the lesser sac and displacing the stomach to the left (Figure 1). An ultrasound scan showed it to consist of cystic and solid elements and it was thought to arise from the tail of the pancreas. A CT scan confirmed the multicystic appearance of the lesion and no liver or lymph node metastases were evident. Fine needle aspiration cytology and CT guided
Figure 1. Barium meal showing displacement ofthe les curvature of the stomach to the left by a retroperitoneal mass
needle biopsies were taken and showed features of a low grade soft tissue tumour, but no specific diagnosis was reached. A digital subtraction coeliac angiogram revealed marked displacement of the superior mesenteric, spleic and portal veins by a large avascular mass.
14IO7"'IV6 0503O8O2/$O2.00/0 o 1991 The Royal Society of Medicine
Journal of the Royal Society of Medicine Volume 84 May 1991
UFigure 2. CT scan of the multicystic mass arising from the tail of the pancreas
At operation the tumour extended from the base of the transverse mesocolon to the fundus of the stomach and appeared to arise from the body and tail ofthe pancreas. The tumour was adherent to, but not obviously infiltrating, the posterior wall of the stomach. There was no evidence of metastatic spread. The lesion was dissected free from the posterior peritoneum and removed with the neck, body and tail of the pancreas and the spleen. There were no postoperative complications. The tumour weighed 3.1 kg and showed the features of a malignant schwannoma. There were up to 3/10 mitoses per high powered field in the cellular areas ofthe tumour, which appeared to be well circumscribed and not invading the pancreas. Nine months after the operation he developed abdominal pain and was found to have multiple secondary deposits in the liver and retroperitoneum. Discussion Retroperitoneal malignant schwannomas in the absence of neurofibromatosis are rare, representing fewer than 2% of all primitive tumours occurring in the region'. Das Gupta2 found only four retroperitoneal tumours in 232 patients with solitary malignant schwannomas not associated with neurofibromatosis. Fewer than 20 cases have been described in detail in the English and 55 cases in the Japanese literature3. Patients typically present with vague upper abdominal pain and a palpable epigastric or left upper quadrant mass4'5. Infection and haemorrhage may complicate or precipitate presentation. Preoperative diagnosis is difficult even with ultrasound and CT scanning6. Seventy-five per cent of malignant retroperitoneal schwannomas have
Testicular lymphoma with pancreatitis
C Ade MB BS1 M Hatton MB BS' P Hamilton Stewart MS FRCS2 B Naylor FRCPath3 D Parker DPhil MRCP1 1Oncology Unit, 2Urology Unit, and 3Department of Histopathology, Bradford Royal Infirmary, Bradford BD9 6RJ Keywords: non-Hodgkin's lymphoma; testis; pancreatitis
A patient with testicular high-grade non-Hodgkin's lymphoma is described who developed obstructive jaundice and
309
cystic changes on imaging often leading to msdiagnosis of pancreatic pseudouy-. Asiratoytbiogy- or trucut biopsy seldom providle sufficent tissue for accurate diagnosis8. These tumours grow to a g s; 8 jitres of fluid was drained from one cystic lesion and narked tumour vascularity has been describedi paticula'in benign schwannomas8. Preoperative airs hy has been , operability and recommended to assess tumour v the origin of tumour blood supply6. The tumour in our g y provided patient was avascular and although a useful information concerning the vaclar ply it was of little diagnostic help. Surgical treatment offers the best prepecteofeur or longterm palliation9. Recurrence, liowever, his usually been reported within 6 mont of rtgical treatment10. The prognosis of malignant retrolpritoeal schwannoma is apparently worse if asoated with nqurofibromatosis2.11. Radiotherapy has been used preoperatively to reduce tumour vascularity, particularly in benign retroperitoneal schwannomas8, but the malignant form' appears to be unresponsive. The use of chemotherapy has been limited and the results inconclusive, however, adriamycin has been the most active drug tried4. The patient is alive at 15 months. References 1 Sordillo PP, Helson L, Hajdu SI, et aL Malignant schwannoma clinical characteristics, survival and response to therapy. Cancer 1981;47:2503-9 2 Das Gupta TK, Brasfield RD. Solitary malignant schwannoma. Ann Surg 1970;171419-28 3 Miyagi T, Shimamura M. Retroperitoneal schwannoma: a report of two cases and review of the literature. Hinoyokika-Kiyo 1986;32:207-14 4 Ball JHS, Sonnendecker EWW, Sevitz H, Murray J. Retroperitoneal malignant schwannoma. S Afr Med J 1987;71:49-52 5 McCarthy S, Duray PH. Giant retroperitoneal neurilemmoma: a rare cause of digestive tract symptoms. J Clin Gastroenterol 1983;5:343-7 6 Pandolfo I, Fiumara F, Chirico G, La Spada F, Caminiti R. Computed tomography in retroperitoneal neurofibrosarcoma (malignant schwannoma). J Comput Assist Tomogr 1983; 7:1119-20 7 Takatera H, Takaha M, Takiuchi H, Ohnishi S, Namiki M, Sonada T. Retrperitoneal schwannoma. Urology 1986;28:529-31 8 Regan JF, Juler GL, Schmutzer KJ. Retroperitoneal neurilemmoma. Am J Surg 1977;134:140-5 9 Moazam F, Rogers BM, Talbert JL. Retroperitoneal malignant schwannoma: a case report. J Pediatr Surg 1983;18:189-92 10 White HR. Survival in malignant schwannoma. Cancer
1971;27:720-9 11 Ghosh BC, Ghosh L, Huvos AG, Fortner JG. Malignant schwannoma. A clinicopathologic study. Cancer 1973;31:184-90
(Accepted 11 December 1990)
pancreatitis due to lymphomatous infiltration of the pancreas. He responded to cytotoxic chemotherapy and remains well one year after treatment.
Case report A 46-year-old man developed swelling and pain in the left testis over a period of 5 weeks. In July 1988, he had a left orchidectomy and histology showed high-grade non-Hodgkin's lymphoma of B cell origin. In November 1988, he developed an illness consisting ofback pain, anorexia and steatorrhoea. He was admitted to hospital with polyuria, polydipsia and glycosuria. On mination he was jaundiced and was tender in the left upper quandrant of the abdomen. Investigations showed blood glucose 13.2 mmol/l, serum amylase 908 IU/1, bilirubin 116 ,mol/l, alkaline phosphatase 418 IU/l and gamma glutamyl transpeptidase 675 IU/1. CT scan of the abdomen showed slight pancreatic enlargement (Figure 1)
0141-0768/91/ 050309-02/$02.00/0 © 1991 The Royal Society of Medicine
