1977, British Journal of Radiology, 50, 364-366
Case reports Multiple polyps of the small intestine: an unusual feature of multiple myeloma By M. Joyce Pais, M.D., M. E. Kricun, M.D., and D. M. Scotti, M.D. Departments of Diagnostic Radiology and Pathology, Hahnemann Medical College and Hospital, 230 North Broad Street, Philadelphia, Pennsylvania 19102 {Received June, 1976 and in revised form January, 1977)
The various syndromes resulting from an abnormal proliferation or neoplasia of mature plasma cells or their immature precursors constitute the plasma cell dyscrasias. These may present as multiple myeloma (plasma cell myeloma), solitary myeloma, extraosseous plasma cell myeloma, or plasma cell leukaemia. We have encountered an unusual case of multiple myeloma in which a patient previously diagnosed by bone biopsy and immunoelectrophoresis, developed multiple non-obstructing polyps of the small intestine and at the oesophagogastric junction. On biopsy, these lesions had the classical histopathology pattern of multiple myeloma. This is the first case of myeloma polyps of the small intestine to be reported in the radiological literature.
myeloma occurring most frequently in the small intestine followed by the stomach, colon and oesophagus. Myeloma rarely occurs in the small intestine. In our review of the literature, only 18 cases were found involving the small intestine and 50% involved the ileum (Esposito and Stout, 1945; Goldstein and Poker, 1966; Ingegno, 1954). Gastrointestinal myeloma may precede, accompany, or follow osseous involvement (Goldstein and Poker, 1966). Clinically, patients usually present with abdominal pain, bleeding and obstruction. Stricture is the predominant radiographic finding. Only two cases of myeloma of the duodenum have been reported (Goldstein and Poker, 1966)—both were obstructing lesions.
CASE REPORT
A 72-year-old white female was admitted in 1972 with a painful mass in the mandible which was diagnosed as myeloma. Two years later, a mass in the right gluteal area appeared. This was biopsied, diagnosed as myeloma, and treated with radiotherapy. In May, 1974, she was readmitted for evaluation of vague abdominal pain, nausea and vomiting, and for treatment of heart failure and hypertension. There was no evidence of gastrointestinal bleeding. Immunoelectrophoresis was compatible with multiple myeloma. Radiographic examination of the upper gastrointestinal tract demonstrated multiple non-obstructing polyps throughout the small bowel (Fig. 1), including the duodenum (Fig. 2), with one rather large polyp at the oesophagogastric junction (Fig. 3). A barium enema was not done. Endoscopy and biopsy were performed and demonstrated the large polyps which proved to be extraosseous myeloma (Fig. 4A and 4B). The patient received a course of cyclophosphamide and vincristine sulphate and was discharged. She was later readmitted to another hospital because of myeloma and congestive heart failure and died. An autopsy was not performed. DISCUSSION
While non-osseous presentation of plasma cell myeloma is unusual, hardly a site exists in which a plasmacytoma has not been reported Oberkircher et al.y (1972). Dolin and Dewar (1956) in reviewing 161 cases between 1905 and 1953 (and adding two of their own) found that a majority occurred in the FIG. 1. oral cavity and upper respiratory tract. The gastroThere are multiple polypoid lesions throughout the small intestinal tract was involved in less than 10% with bowel measuring 0.8 to 1.2 cm (arrows). 364
MAY
Case reports
FIG. 2. Several polypoid non-obstructing myeloma masses are present in the second and third portions of the duodenum (arrows).
FIG. 3. There is a polypoid lesion at the oesophagogastric region measuring 2.5 cm (arrow). FIG. 4. (A) Myeloma polyp as seen through the fibre-endoscope (P). (B) Microscopic section of a duodenal polyp showing nests of small dark cells consisting of plasma cells and immature cells (plasma), some containing double nuclei. These cells are consistent with myeloma cells.
365
1977
1977, British Journal of Radiology, 50, 366-369 Case reports
Although rare, extraosseous myeloma should be considered in the differential diagnosis of multiple polyps in the small intestine.
cytoma of the jejunum. American Journal of Pathology, 53, 33-39. GOLDSTEIN, W. B., and POKER, N., 1966. Multiple myeloma
involving the gastrointestinal tract. Gastroenterology, 51, 87-93. INGEGNO, A. P., 1954. Plasmacytoma of the gastrointestinal
tract: Report of a case involving the jejunum and review of the literature. Gastroenterology, 26, 89-102.
REFERENCES DOLIN, S., and DEWAR, J. P., 1956. Extramedullary plasma-
OBERKIRCHER, P. E., MILLER, W. T., and ARGER, P. H.,
cytoma. American Journal of Pathology, 32, 83-100.
1972. Non-osseous presentation of plasma cell myeloma. Radiology, 104, 515-520.
ESPOSITO, J. J., and STOUT, A. P., 1945. Multiple plasma-
Caroli's disease: an ultrasonic diagnosis By E. M. Bass, M.B.Ch.B., M.Med. (Rad.D.), D.M.R.D., F.R.C.R., M. R. Funston, M.B.B.Ch., D.M.R.D., F.R.C.R., and M. I. Shaft, M.B.B.Ch., D.M.R.D., F.R.C.R. Department of Radiology, Groote Schuur Hospital, Observatory, Cape Town 7925, South Africa (Received October, 1976)
Congenital dilatation of the intrahepatic bile ducts (Caroli's disease) was first reported in 1958 (Caroli et al.) and is usually diagnosed late in the patient's illness and seldom before operation (Watts et al., 1974). Goddard (1974) could only find 16 previous case reports of the disease which attests to its rarity. Caroli (1968) has classified congenital intrahepatic cystic dilatation into two groups: first, a pure form not associated with hepatic cirrhosis or portal hypertension and second, a type associated with congenital hepatic fibrosis. There is probably some overlap but Watts et al. (1974) consider most cases belong to the first group. The radiological diagnosis of Caroli's disease has, in the past, proven difficult. None of the 15 cases collected by Mujahed et al. (1971) were diagnosed before surgery. With improved modern techniques in organ visualization, the pre-operative diagnosis has become easier and we report the following case which was confidently diagnosed by ultrasonography and confirmed by transhepatic cholangiography, thus rendering invaluable assistance to the surgeon.
In February 1976 her jaundice and rigors recurred. She was now pyrexial and her liver was enlarged by 3 cm. Her total bilirubin was 9.3 mg%. An isotopic technetium sulphur colloid scan showed a cold area in the A.I. portion of the right lobe. A Rose-Bengal scan was not performed. Her intravenous pyelogram was normal with no evidence of renal tubular ectasia. Longitudinal and transverse grey-scale ultrasonograms, at high and low gain, were done with Diasonograph NE 4102 scan convertor, using a 2.5 MHz transducer. Four transonic lesions, varying from 2 to 5 cm in diameter, were found in the region of the porta hepatis (Fig. 1). Their cystic nature was confirmed by the absence of internal echoes at high and low gain and clear definition of their posterior walls. Their position was marked on the skin surface and longitudinal, sagittal scans were next done through the plane of these marks. The largest cystic structure was 5 cm in diameter and 12 cm in length (Fig. 2). An
CASE REPORT
A female of 19 years was first admitted to hospital in December 1975 because of increasing jaundice and rigors. Her clinical history was unremarkable except for her occupation as an assistant in a hospital sterilizing department and handling liver biopsy packs. Her total bilirubin was 5 mg% (conjugated level 3.8 mg%) and her alkaline phosphatase was 132 international units. She was thought to have infective hepatitis but her jaundice never settled and a liver biopsy was compatible FIG.1. with cholangitis and biliary stasis. Her jaundice subsequently decreased, her symptoms improved and she was Transverse scan through the region of the porta hepatis showing four transonic structures. discharged. 366
Case reports Multiple polyps of the small intestine: an unusual feature of multiple myeloma By M. Joyce Pais, M.D., M. E. Kricun, M.D., and D. M. Scotti, M.D. Departments of Diagnostic Radiology and Pathology, Hahnemann Medical College and Hospital, 230 North Broad Street, Philadelphia, Pennsylvania 19102 {Received June, 1976 and in revised form January, 1977)
The various syndromes resulting from an abnormal proliferation or neoplasia of mature plasma cells or their immature precursors constitute the plasma cell dyscrasias. These may present as multiple myeloma (plasma cell myeloma), solitary myeloma, extraosseous plasma cell myeloma, or plasma cell leukaemia. We have encountered an unusual case of multiple myeloma in which a patient previously diagnosed by bone biopsy and immunoelectrophoresis, developed multiple non-obstructing polyps of the small intestine and at the oesophagogastric junction. On biopsy, these lesions had the classical histopathology pattern of multiple myeloma. This is the first case of myeloma polyps of the small intestine to be reported in the radiological literature.
myeloma occurring most frequently in the small intestine followed by the stomach, colon and oesophagus. Myeloma rarely occurs in the small intestine. In our review of the literature, only 18 cases were found involving the small intestine and 50% involved the ileum (Esposito and Stout, 1945; Goldstein and Poker, 1966; Ingegno, 1954). Gastrointestinal myeloma may precede, accompany, or follow osseous involvement (Goldstein and Poker, 1966). Clinically, patients usually present with abdominal pain, bleeding and obstruction. Stricture is the predominant radiographic finding. Only two cases of myeloma of the duodenum have been reported (Goldstein and Poker, 1966)—both were obstructing lesions.
CASE REPORT
A 72-year-old white female was admitted in 1972 with a painful mass in the mandible which was diagnosed as myeloma. Two years later, a mass in the right gluteal area appeared. This was biopsied, diagnosed as myeloma, and treated with radiotherapy. In May, 1974, she was readmitted for evaluation of vague abdominal pain, nausea and vomiting, and for treatment of heart failure and hypertension. There was no evidence of gastrointestinal bleeding. Immunoelectrophoresis was compatible with multiple myeloma. Radiographic examination of the upper gastrointestinal tract demonstrated multiple non-obstructing polyps throughout the small bowel (Fig. 1), including the duodenum (Fig. 2), with one rather large polyp at the oesophagogastric junction (Fig. 3). A barium enema was not done. Endoscopy and biopsy were performed and demonstrated the large polyps which proved to be extraosseous myeloma (Fig. 4A and 4B). The patient received a course of cyclophosphamide and vincristine sulphate and was discharged. She was later readmitted to another hospital because of myeloma and congestive heart failure and died. An autopsy was not performed. DISCUSSION
While non-osseous presentation of plasma cell myeloma is unusual, hardly a site exists in which a plasmacytoma has not been reported Oberkircher et al.y (1972). Dolin and Dewar (1956) in reviewing 161 cases between 1905 and 1953 (and adding two of their own) found that a majority occurred in the FIG. 1. oral cavity and upper respiratory tract. The gastroThere are multiple polypoid lesions throughout the small intestinal tract was involved in less than 10% with bowel measuring 0.8 to 1.2 cm (arrows). 364
MAY
Case reports
FIG. 2. Several polypoid non-obstructing myeloma masses are present in the second and third portions of the duodenum (arrows).
FIG. 3. There is a polypoid lesion at the oesophagogastric region measuring 2.5 cm (arrow). FIG. 4. (A) Myeloma polyp as seen through the fibre-endoscope (P). (B) Microscopic section of a duodenal polyp showing nests of small dark cells consisting of plasma cells and immature cells (plasma), some containing double nuclei. These cells are consistent with myeloma cells.
365
1977
1977, British Journal of Radiology, 50, 366-369 Case reports
Although rare, extraosseous myeloma should be considered in the differential diagnosis of multiple polyps in the small intestine.
cytoma of the jejunum. American Journal of Pathology, 53, 33-39. GOLDSTEIN, W. B., and POKER, N., 1966. Multiple myeloma
involving the gastrointestinal tract. Gastroenterology, 51, 87-93. INGEGNO, A. P., 1954. Plasmacytoma of the gastrointestinal
tract: Report of a case involving the jejunum and review of the literature. Gastroenterology, 26, 89-102.
REFERENCES DOLIN, S., and DEWAR, J. P., 1956. Extramedullary plasma-
OBERKIRCHER, P. E., MILLER, W. T., and ARGER, P. H.,
cytoma. American Journal of Pathology, 32, 83-100.
1972. Non-osseous presentation of plasma cell myeloma. Radiology, 104, 515-520.
ESPOSITO, J. J., and STOUT, A. P., 1945. Multiple plasma-
Caroli's disease: an ultrasonic diagnosis By E. M. Bass, M.B.Ch.B., M.Med. (Rad.D.), D.M.R.D., F.R.C.R., M. R. Funston, M.B.B.Ch., D.M.R.D., F.R.C.R., and M. I. Shaft, M.B.B.Ch., D.M.R.D., F.R.C.R. Department of Radiology, Groote Schuur Hospital, Observatory, Cape Town 7925, South Africa (Received October, 1976)
Congenital dilatation of the intrahepatic bile ducts (Caroli's disease) was first reported in 1958 (Caroli et al.) and is usually diagnosed late in the patient's illness and seldom before operation (Watts et al., 1974). Goddard (1974) could only find 16 previous case reports of the disease which attests to its rarity. Caroli (1968) has classified congenital intrahepatic cystic dilatation into two groups: first, a pure form not associated with hepatic cirrhosis or portal hypertension and second, a type associated with congenital hepatic fibrosis. There is probably some overlap but Watts et al. (1974) consider most cases belong to the first group. The radiological diagnosis of Caroli's disease has, in the past, proven difficult. None of the 15 cases collected by Mujahed et al. (1971) were diagnosed before surgery. With improved modern techniques in organ visualization, the pre-operative diagnosis has become easier and we report the following case which was confidently diagnosed by ultrasonography and confirmed by transhepatic cholangiography, thus rendering invaluable assistance to the surgeon.
In February 1976 her jaundice and rigors recurred. She was now pyrexial and her liver was enlarged by 3 cm. Her total bilirubin was 9.3 mg%. An isotopic technetium sulphur colloid scan showed a cold area in the A.I. portion of the right lobe. A Rose-Bengal scan was not performed. Her intravenous pyelogram was normal with no evidence of renal tubular ectasia. Longitudinal and transverse grey-scale ultrasonograms, at high and low gain, were done with Diasonograph NE 4102 scan convertor, using a 2.5 MHz transducer. Four transonic lesions, varying from 2 to 5 cm in diameter, were found in the region of the porta hepatis (Fig. 1). Their cystic nature was confirmed by the absence of internal echoes at high and low gain and clear definition of their posterior walls. Their position was marked on the skin surface and longitudinal, sagittal scans were next done through the plane of these marks. The largest cystic structure was 5 cm in diameter and 12 cm in length (Fig. 2). An
CASE REPORT
A female of 19 years was first admitted to hospital in December 1975 because of increasing jaundice and rigors. Her clinical history was unremarkable except for her occupation as an assistant in a hospital sterilizing department and handling liver biopsy packs. Her total bilirubin was 5 mg% (conjugated level 3.8 mg%) and her alkaline phosphatase was 132 international units. She was thought to have infective hepatitis but her jaundice never settled and a liver biopsy was compatible FIG.1. with cholangitis and biliary stasis. Her jaundice subsequently decreased, her symptoms improved and she was Transverse scan through the region of the porta hepatis showing four transonic structures. discharged. 366
