Case reports

1990, The British Journal of Radiology, 63, 814-816 Discussion

Lymphangiomas are classified histologically into simple, cavernous and cystic, according to the size of the dilated lymphatics. Cystic lymphangiomas are unilocular or multilocular masses containing serous or chylous fluid. The cyst wall and septum consist of fibrous tissues, lymphatic tissues, vessels and smooth muscles with an inner surface of endothelial cells. Cystic lymphangiomas arise as the result of congenital malformation of the lymphatic system (Godart, 1966) and are considered to be lymphatic hamartomas. There is a lack of adequate drainage from the sequestrated lymphatic vessels to the venous system because of insufficiency or atresia of the afferent channels. About 50% of these lesions are present at birth and up to 90% are evident by 2 years of age. Lymphangiomas enlarge during the early growth of the infant. Ninety five per cent of lesions arise in the neck and the axilla. The rest arise in various sites. In the literature, we have found 15 cases of scrotal lymphangioma (MacMillan et al, 1984). We have also found six lymphangiomas of the inguinal area. In three cases, children of 70 days, 5 months and 3 years developed lymphangiomas mimicking an inguinal hernia (Hoffman, 1965; Kafka & Novak, 1970; Aboulola et al, 1975). In two further cases, children of 2\ years and 2 years and 4 months, the lymphangioma appeared as an inguinal/abdominal tumour (Aboulola et al, 1975). The case described by Singh et al (1975) contains no clinical information. On CT or ultrasound, it is usual to find cystic areas with multiple septa. In our case, hemorrhage inside the cystic area explained thefluidlevels.

Cystic lymphangiomas seldom cause acute clinical symptoms, but these may manifest themselves when the retroperitoneal cystic lymphangiomas are large enough to cause pressure on adjacent structures, such as the bowel and the ureters, or when they are complicated by infection, hemorrhage, or rupture. Conclusion

We report a case of a rare inguinal lymphangioma where the diagnosis was facilitated pre-operatively by ultrasound and CT. Acknowledgments The authors would like to thank Dr P. B. Guyer, Royal South Hants Hospital, Southampton, for his help. References ABOULOLA, M., BOUKHELOUA, A., DANIEL, F. & BOUHADAFF,

A., 1975. Lymphangiomes kystiques abdominaux chez l'enfant. Chirurgie, 101, 852-857. GODART, S., 1966. Embryological significance of lymphangioma. Archives of Disease in Childhood, 41, 204-205. HOFFMAN, E., 1965. Multicystic retroperitoneal lymphangioma presenting as an indirect inguinal hernia in a newborn. American Surgeon, 31, 525-531. KAFKA, V. & NOVAK, K., 1970. Multicystic retroperitoneal

lymphangioma in an infant appearing as an inguinal hernia. Journal of Pediatric Surgery, 5, 573. MACMILLAN, R. W., MACDONALD, B. R. & ALPHERN, H. D.,

1984. Scrotal lymphangioma. Urology, 23, 79-80. SINGH, S.,

BABOO, M.

L. & PATHAK, I. C ,

1971.

Cystic

lymphangioma in children; report of 32 cases including lesions at rare site. Surgery, 69, 947-951.

Unusual presentation of non-Hodgkin's lymphoma of the small bowel By D. A. Nicholson, BMedSci, BM, BS, FRCR Department of Radiology, University Hospital of South Manchester, Nell Lane, West Didsbury, Manchester M20 8LR (Received February 1990 and in revised form March 1990)

Small bowel non-Hodgkins lymphoma (NHL) usually present with abdominal pain (72%), vomiting and diarrhoea (28%); however, gastrointestinal (GI) bleeding is an uncommon mode of presentation (4%) (Makepeace et al, 1987). A case is presented where a patient with coexisting small bowel NHL and an abdominal aortic aneurysm presented with clinical symptoms suggestive of an aortoenteric fistula. The mode of presentation for NHL is unusual, with computed tomography (CT) playing a significant role in the diagnosis and management. Case report A 75-year-old man presented with a 1-month history of intermittent episodes of non-colicky upper abdominal pain,

814

mild weight loss, malaise, and black stools. Past medical history was unremarkable. On examination he was anaemic with a pulsatile abdominal mass. Investigation revealed a haemoglobin of 4.9 g/dl, with normal urea, electrolytes, liver function tests and white count. Severe melaena with positive occult bloods was confirmed. An abdominal ultrasound scan showed the presence of an 8 cm abdominal aortic aneurysm, with no evidence of leakage. Initially the patient was transfused with 6 units of blood. An urgent fibre-optic gastroscopy revealed no gastric or proximal duodenal abnormality. A presumptive diagnosis of aortoenteric fistula was made, on the clinical triad of upper GI bleeding, abdominal pain and abdominal aortic aneurysm (Ferguson & Arden, 1966). An abdominal CT scan confirmed a large infrarenal aortic aneurysm which, although thin walled and close to the duodenum, showed no leak or erosion into bowel. In the upper

The British Journal of Radiology, October 1990

Case reports aneurysm no aortoenteric fistula was identified. A routine aneurysm repair was performed with a Dacron tube graft. Histology confirmed the neoplasms as high grade diffuse nonHodgkin's lymphoma extending through the bowel wall and involving peri-intestinal fat. No involved lymph nodes were identified.

Discussion

Figure 1. Computed tomographic scan during dynamic intravenous contrast infusion (level of lower poles of kidneys). Thin-walled aneurysm with mural thrombus (small arrow). Circumferential thickening of a small bowel loop is seen (curved arrow), with further dilated loops of small bowel (open arrow).

abdomen there were multiple loops of fluid-filled dilated small bowel. In addition, two separate abnormal areas of bowel were demonstrated; one with circumferential thickening of the bowel wall (Fig. 1), the second showing a lobular mass ( 4 x 3 cm) with proximal small bowel dilatation (Fig. 2). The liver and spleen were normal with no evidence of adenopathy. At laparotomy, the proximal 1.5 m of small bowel were dilated leading to a 4 cm segment of intussuscepted nodular, haemorrhagic bowel. A further 3 cm long annular tumour was found 40 cm distal to the first lesion. No further intraperitoneal disease was seen. The diseased small bowel was resected and an end to end anastomosis performed. On inspection of the aortic

Figure 2. Computed tomographic scan during dynamic intravenous contrast infusion (level of iliac blades). A separate lobular mass is seen in the small bowel (curved arrow).

Vol. 63, No. 754

Primary lymphoma is one of the commonest tumours of the small bowel accounting for about 40% of primary malignant neoplasms (Good, 1963). The most common site of involvement is the distal ileum, because of its abundance of lymphatic tissue; the duodenum is rarely involved (Brady & Ashell, 1980). Non-Hodgkin's lymphoma has a tendency to involve extranodal areas as its primary site, with the GI tract being the commonest involved organ (Freeman et al, 1972). However, 5070% of patients with disseminated lymphoma will have incidental GI involvement (Rosenburgh et al, 1961). Mesenteric involvement and adenopathy is found in 50% of NHL, but only 4% of those with Hodgkin's disease (Spjut & Navarrtte, 1978). Multifocal tumours occur in 30% (Zornoza & Dodd, 1980), tending to be in the same organ (Green et al, 1979), as in this case. According to most reports, surgery alone can be curative if the tumour is resectable (Lim et al, 1977). However, GI lymphoma is usually of an aggressive histology which adversely affects the prognosis: 5-year survival is 40% (Lim et al, 1977; Paulson et al, 1983). In addition, surgery carries a significant risk in the elderly, with a reported operative mortality of 9-19% (Maor et al, 1984). Gastrointestinal bleeding, along with prodromal symptoms, is relatively uncommon in small bowel NHL, although abdominal pain is invariable. A small proportion present with malabsorption (Isaacson & Wright, 1978). A striking feature is the high incidence of perforation in contrast to other more common small bowel pathology such as Crohn's disease, with which confusion is common (Green et al, 1979). Further diagnostic difficulty occurs in distinguishing GI-NHL from carcinoma and carcinoid, although lymphomas often involve longer segments and are frequently multiple. The most common radiological appearance of small bowel lymphoma is luminal narrowing, including stricture formation (Gourtsoyiannis & Nolan, 1988), intussuseption is uncommon. Other appearances include polypoid, infiltrating, ulceration, cavitation, thickened valvulae, multiple nodules, mesenteric masses and aneurysmal dilatation. The CT manifestations again exhibit a broad spectrum of radiological changes which depend on the underlying pattern of pathological involvement. Marked thickening of the bowel may be the only abnormality. Mesenteric involvement produces typical irregular lobulated masses that tend to displace bowel and may cause obstruction. Areas of reduced attenuation within the mesenteric masses occasionally occur owing to tumour necrosis. Because of non-specfic presentation of small bowel lymphoma, the tumour is rarely diagnosed prior to 815

Case reports

1990, The British Journal of Radiology, 63, 816-818

surgery (Green et al, 1979). Although an aortoenteric fistula was clinically suspected in this patient, the CT revealed two abnormal areas of bowel at different sites, with associated obstruction. This raised the suspicion of a co-existing NHL with the aortic aneurysm, giving the opportunity to perform a formal staging laparotomy, adding valuable additional information in planning further treatment.

ISSACSON, P. & WRIGHT, D. H., 1978. Intestinal lymphomas

associated with malabsorption. Lancet, i, 67-70. LIM, F. E., HAETMAN, A. S., TAN, E. G., CADY, B. & MEISSNER,

W. A., 1977. Factors in the prognosis of gastric lymphoma. Cancer, 39, 1715-1720. MAKEPEACE, A. R., FERMONT, D. C. & BENNETT, M. H., 1987.

Gastrointestinal non-Hodgkin's Radiology, 38, 609-614.

lymphoma.

Clinical

MAOR, M. H., MADDUX, B., OSBOURNE, B. M., FULLER, L. M., SULLIVAN, J. A. & NELSON, R. S., 1984. Stages IE and HE

References BRADY, L. W. & ASHELL, S. O., 1980. Malignant lymphoma of

the gastointestinal tract. Radiology, 137, 291. FERGUSON, J. P. & ARDEN,

1979. The presentation of gastrointestinal lymphoma: study of a population. British Journal of Surgery, 66, 798-801.

M. J.,

non-Hodgkin's lymphoma of the stomach. Cancer, 54, 2330-2337.

1966. Gastrointestinal

PAULSON, S., SHEEHAN, R. G. & STONE, M. J., 1983. Large cell

haemorrhage secondary to rupture of the aorta. Archives of Internal Medicine, 117, 133-140.

lymphomas of the stomach: improved prognosis with the resection of all intrinsic gastrointestinal disease. Journal of Clinical Oncology, 1, 263-269.

FREEMAN, C , BERG, J. W. & CUTLER, S. J., 1972. Occurence

and prognosis of extranodal lymphomas. Cancer, 29, 252-260. GOOD, C. A., 1963. Tumours of the small intestine. American Journal of Roentgenology, 89, 685-705. GOURTSOYIANNIS, N. C. & NOLAN, D. J., 1988. Lymphoma of

the small bowel: radiological Radiology, 39, 639 645.

appearances.

Clinical

GREEN, J. A., DAWSON, A. A., JONES, P. F. & BRUNT, P. W.,

ROSENBERG, S. A., DIAMOND, H. D., JASLOWITZ, B. & CRAVER,

L. F., 1961. Lymphosarcoma: a review of 1209 cases. Medicine, 40, 31-84. SPJUT, A. J. & NAVARETTE, A., 1978. Alimentary

Tract

Roentgenology (C. V. Mosby, St Louis), p. 787. ZORNOZA,

J. & DODD,

gastrointestinal 272-287.

tract.

G.

D.,

Seminars

1980. Lymphoma

of the

in Roentgenology, 15,

Ultrasound appearances of extramedullary haematopoiesis in the liver and spleen By M. J . Bradley, M B , ChB, FRCR and C. Metreweli, FRCR, FRCP Department of Radiodiagnosis and Organ Imaging, Prince of Wales Hospital, Shatin, Hong Kong

(Received December 1989)

Extramedullary haematopoiesis is an uncommon finding in the adult patient. Many sites have been described, the liver and spleen being more common. The ultrasound appearances have not been well documented. Two cases of extramedullary haematopoiesis in the liver and spleen are presented.

size, and contained one echogenic mass similar to those in the liver. No other abnormality was shown in the remainder of the abdomen and pelvis. The most likely diagnosis for these lesions was metastatic

Case reports Case 1 A 58-year-old man presented with a history of a cough, haemoptysis, dyspnoea and weight loss. On examination he had finger clubbing, no significant chest signs and hepatomegaly. A bronchial neoplasm was suspected clinically and the initial chest radiograph demonstrated a right lung mass adjacent to the hilum. Full blood count was normal and the erythrocyte sedimentation rate was raised at 52 mm/h. Bronchoscopy revealed extrinsic compression of the right upper lobe bronchus but biopsy was unsuccessful. Ultrasound examination of the abdomen demonstrated multiple echogenic lesions in an enlarged liver (Fig. 1). Some of these lesions contained central echo-poor areas. The spleen was of normal Address for correspondence: University Department of Radiodiagnosis, Royal Liverpool Hospital, Prescot Street, Liverpool L7 8XP.

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Figure 1. Longitudinal section of the right lobe of liver showing several echogenic foci, one with central necrosis.

The British Journal of Radiology, October 1990

Unusual presentation of non-Hodgkin's lymphoma of the small bowel.

Case reports 1990, The British Journal of Radiology, 63, 814-816 Discussion Lymphangiomas are classified histologically into simple, cavernous and c...
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