1979, British Journal of Radiology, 52, 326-327 Case reports FIG. 2. Extensive paravertebral ossification and disc space narrowing. Following chemotherapy the margins of the vertebral bodies are clearly defined.
Pseudomonas osteomyelitis is recognized as occurring in drug addicts and also as a feature of direct spread from an adjacent site of infection (Salahuddin et al., 1973). Haematogenous spread of pseudomonas in the non-addict must be extremely rare. REFERENCES LOWMAN, R. F., ROBINSON, F., 1966. Progressive vertebral
interspace changes following lumbar disc surgery. American Journal of Roentgenology, 97, 664-671. PILGAARD, S., AARHUS, N., 1969. Discitis. Closed space infection following removal of lumbar intervertebral disc. Journal of Bone and Joint Surgery, 51 h, 713-716. SALAHUDDIN, N. I., MADHAVAN, T., FISHER, E. J., COX, F., QUINN, E. L., EYLER, W. R., 1973. Pseudomonas
osteomyelitis. Radiologic features. Radiology, 109, 41-47.
The use of percutaneous transhepatic cholangiography in a case of embryonal rhabdomyosarcoma By P. M. Cannon, M.B., D. A. Legge, M.B., F.R.C.R. Department of Radiology, Mater Hospital, Dublin 7 and B. O'Donnell, M.Ch., F.R.C.S. Department of Surgery, Our Lady's Hospital for Sick Children, Dublin 12 (Received August, 1978)
Embryonal rhabdomyosarcoma (sarcoma botryoides) of the bile ducts is a tumour of mesodermal origin characterized by the growth of large polypoid mass lesions (Ackerman and Rosai, 1974). This paper describes a case of embryonal rhabdomyosarcoma arising from the common hepatic duct of a child and
emphasizes the role of percutaneous transhepatic cholangiography in the management of the patient. CASE REPORT
A four and a half-year-old female was admitted to a fever hospital with an acute onset of jaundice. She had a liver palpable two centimetres below the costal margin, and a
326
APRIL 1979
Case reports portion of the hepatic duct and extending behind the head of the pancreas. The entire mass was excised and the common hepatic duct anastomosed end to side to a loop of jejunum. There were no obvious secondary deposits at the time of surgery. On examination the tumour measured 1 2 x 7 x 7 cm and was diagnosed histologically as an embryonal rhabdomyosarcoma on the findings of typical spindle shaped tumour cells with characteristic cross striations. Since the operation the patient has been given combined chemotherapy with vincristine, adriomycin, and cyclophosphamide at two weekly intervals. She has no evidence for recurrence so far, 18 months since the operation. DISCUSSION
FIG.1.
Transhepatic cholangiogram. Arrow points to totally obstructed common hepatic duct. temperature of 37.8CC. Examination was otherwise normal. Haemoglobin was 13.4 gm/dl (13.4 gm/100 ml), white cell count 13.7 X 109/1 (13,700/mm3), serum bilirubin 87 /*mol/1 (5.1 mg/d1), direct 53 /xmol/1 (3.1 mg/dl), SGOT 118 IU/1, SGPT 156 IU/1, alkaline phosphatase 2178 IU/1. Serum protein values were normal. The patient's jaundice increased and this was associated with a rise in both serum transaminase and alkaline phosphatase levels. Sixteen days after admission treatment with corticosteroids resulted in significant clinical improvement and a fall in the serum bilirubin value to 34 /umol/1 (2.0 mg/dl). However, there was a progressive rise in serum globulin to 51 g/1 (5.1 g/dl). The patient was discharged after nine weeks of hospitalization with a diagnosis of chronic active hepatititis. Four weeks after discharge she was admitted to hospital with a history of increasing jaundice, pruritis, right hypochondrial pain, pale stools and dark urine. Her liver was now palpable five centimetres below the costal margin. Serum bilirubin was 215 ju.mol/1 (12.6 mg/dl), alkaline phosphatase 88 IU/1, SGOT 158 IU/1, SGPT 264 IU/1, serum albumin 26 g/1 (3.6 g/dl), serum globulin 43 g/1 (4.3 g/dl) with raised a1 and a.2 fractions. Percutaneous transhepatic cholangiography was performed and demonstrated complete obstruction of the common hepatic duct (Fig. 1). Rhabdomyosarcoma of the common bile duct was the only diagnosis suggested on these appearances. At surgical exploration a large lobulated mass was shown involving the common hepatic duct, common bile duct, a
Embryonal rhabdomyosarcoma of the bile ducts is a rare lesion, first documented by Wilks and Moxon in 1875. Since then 28 cases have been described in children whose ages ranged from 16 months to 11 years. The tumour has been reported in 16 females and 12 males and mean survival from time of presentation has been 5.3 months. The longest survival recorded was 16 months. Metastatic spread to lung, skeleton and lymph nodes may occur, but frequently death is due to direct extension of the tumour (Majmudar and Kumar, 1976; Sarrazin et al., 1977). Although embryonal rhabdomyosarcoma is sensitive to irradiation and chemotherapy, surgical excision is necessary to achieve a cure and therefore prompt diagnosis is essential. However, most of the cases reported were thought to have infectious hepatitis and were treated as such for a month or more (Majmudar and Kumar, 1976). As our case illustrates this must be due partially to a low index of suspicion for such a tumour, and partially to a lack of a clearcut obstructive pattern in the initial clinical history and biochemical pattern. In such instances embryonal rhabdomyosarcoma should be included in the differential diagnosis. Percutaneous transhepatic cholangiographic studies should be undertaken at an early stage of investigations if early diagnosis is to be achieved and definitive surgical treatment made possible. REFERENCES ACKERMAN, L. V., and ROSAI, J., 1974. Surgical Pathology,
5th Edition, pp. 1146-9. (C. V. Mosby Company, St. Louis.) MAJMUDAR, B., and KUMAR, V. S., 1976. Embryonal
rhabdomyosarcoma (sarcoma botryoides) of the common bile duct: a case report. Human Pathology, 7, 6, 705-8. SARRAZIN, R., DYON, J. F., BRABANT, A., PONT, J., and
GOUT, J. P., 1977. Sarcoma botryoide du choledoque: reflexions a propos d'un cas et revue de la litterature. Medecine et Chirurgie Digestives, 6, 45-50. WILKS, S., and MOXON, W., 1875. Lectures on Pathological
327
Anatomy. 2nd Edition, Ed., Longman, Brown, Green, Roberts and Company, London, p. 465.
Pseudomonas osteomyelitis is recognized as occurring in drug addicts and also as a feature of direct spread from an adjacent site of infection (Salahuddin et al., 1973). Haematogenous spread of pseudomonas in the non-addict must be extremely rare. REFERENCES LOWMAN, R. F., ROBINSON, F., 1966. Progressive vertebral
interspace changes following lumbar disc surgery. American Journal of Roentgenology, 97, 664-671. PILGAARD, S., AARHUS, N., 1969. Discitis. Closed space infection following removal of lumbar intervertebral disc. Journal of Bone and Joint Surgery, 51 h, 713-716. SALAHUDDIN, N. I., MADHAVAN, T., FISHER, E. J., COX, F., QUINN, E. L., EYLER, W. R., 1973. Pseudomonas
osteomyelitis. Radiologic features. Radiology, 109, 41-47.
The use of percutaneous transhepatic cholangiography in a case of embryonal rhabdomyosarcoma By P. M. Cannon, M.B., D. A. Legge, M.B., F.R.C.R. Department of Radiology, Mater Hospital, Dublin 7 and B. O'Donnell, M.Ch., F.R.C.S. Department of Surgery, Our Lady's Hospital for Sick Children, Dublin 12 (Received August, 1978)
Embryonal rhabdomyosarcoma (sarcoma botryoides) of the bile ducts is a tumour of mesodermal origin characterized by the growth of large polypoid mass lesions (Ackerman and Rosai, 1974). This paper describes a case of embryonal rhabdomyosarcoma arising from the common hepatic duct of a child and
emphasizes the role of percutaneous transhepatic cholangiography in the management of the patient. CASE REPORT
A four and a half-year-old female was admitted to a fever hospital with an acute onset of jaundice. She had a liver palpable two centimetres below the costal margin, and a
326
APRIL 1979
Case reports portion of the hepatic duct and extending behind the head of the pancreas. The entire mass was excised and the common hepatic duct anastomosed end to side to a loop of jejunum. There were no obvious secondary deposits at the time of surgery. On examination the tumour measured 1 2 x 7 x 7 cm and was diagnosed histologically as an embryonal rhabdomyosarcoma on the findings of typical spindle shaped tumour cells with characteristic cross striations. Since the operation the patient has been given combined chemotherapy with vincristine, adriomycin, and cyclophosphamide at two weekly intervals. She has no evidence for recurrence so far, 18 months since the operation. DISCUSSION
FIG.1.
Transhepatic cholangiogram. Arrow points to totally obstructed common hepatic duct. temperature of 37.8CC. Examination was otherwise normal. Haemoglobin was 13.4 gm/dl (13.4 gm/100 ml), white cell count 13.7 X 109/1 (13,700/mm3), serum bilirubin 87 /*mol/1 (5.1 mg/d1), direct 53 /xmol/1 (3.1 mg/dl), SGOT 118 IU/1, SGPT 156 IU/1, alkaline phosphatase 2178 IU/1. Serum protein values were normal. The patient's jaundice increased and this was associated with a rise in both serum transaminase and alkaline phosphatase levels. Sixteen days after admission treatment with corticosteroids resulted in significant clinical improvement and a fall in the serum bilirubin value to 34 /umol/1 (2.0 mg/dl). However, there was a progressive rise in serum globulin to 51 g/1 (5.1 g/dl). The patient was discharged after nine weeks of hospitalization with a diagnosis of chronic active hepatititis. Four weeks after discharge she was admitted to hospital with a history of increasing jaundice, pruritis, right hypochondrial pain, pale stools and dark urine. Her liver was now palpable five centimetres below the costal margin. Serum bilirubin was 215 ju.mol/1 (12.6 mg/dl), alkaline phosphatase 88 IU/1, SGOT 158 IU/1, SGPT 264 IU/1, serum albumin 26 g/1 (3.6 g/dl), serum globulin 43 g/1 (4.3 g/dl) with raised a1 and a.2 fractions. Percutaneous transhepatic cholangiography was performed and demonstrated complete obstruction of the common hepatic duct (Fig. 1). Rhabdomyosarcoma of the common bile duct was the only diagnosis suggested on these appearances. At surgical exploration a large lobulated mass was shown involving the common hepatic duct, common bile duct, a
Embryonal rhabdomyosarcoma of the bile ducts is a rare lesion, first documented by Wilks and Moxon in 1875. Since then 28 cases have been described in children whose ages ranged from 16 months to 11 years. The tumour has been reported in 16 females and 12 males and mean survival from time of presentation has been 5.3 months. The longest survival recorded was 16 months. Metastatic spread to lung, skeleton and lymph nodes may occur, but frequently death is due to direct extension of the tumour (Majmudar and Kumar, 1976; Sarrazin et al., 1977). Although embryonal rhabdomyosarcoma is sensitive to irradiation and chemotherapy, surgical excision is necessary to achieve a cure and therefore prompt diagnosis is essential. However, most of the cases reported were thought to have infectious hepatitis and were treated as such for a month or more (Majmudar and Kumar, 1976). As our case illustrates this must be due partially to a low index of suspicion for such a tumour, and partially to a lack of a clearcut obstructive pattern in the initial clinical history and biochemical pattern. In such instances embryonal rhabdomyosarcoma should be included in the differential diagnosis. Percutaneous transhepatic cholangiographic studies should be undertaken at an early stage of investigations if early diagnosis is to be achieved and definitive surgical treatment made possible. REFERENCES ACKERMAN, L. V., and ROSAI, J., 1974. Surgical Pathology,
5th Edition, pp. 1146-9. (C. V. Mosby Company, St. Louis.) MAJMUDAR, B., and KUMAR, V. S., 1976. Embryonal
rhabdomyosarcoma (sarcoma botryoides) of the common bile duct: a case report. Human Pathology, 7, 6, 705-8. SARRAZIN, R., DYON, J. F., BRABANT, A., PONT, J., and
GOUT, J. P., 1977. Sarcoma botryoide du choledoque: reflexions a propos d'un cas et revue de la litterature. Medecine et Chirurgie Digestives, 6, 45-50. WILKS, S., and MOXON, W., 1875. Lectures on Pathological
327
Anatomy. 2nd Edition, Ed., Longman, Brown, Green, Roberts and Company, London, p. 465.
