Clin. Radiol. (1977) 28, 327-331 LYMPHOGRAPHY IN ALVEOLAR SOFT PART SARCOMA C. A. PARSONS and J. S. MacDONALD From the Diagnostic X-ray Departments o f the Royal Marsden Hospital, Sutton, Surrey, and the Royal Air Force Hospital, Halton, Aylesbury, Bucks. Alveolar soft part sarcoma is a rare tumour found in musculofascial planes, most often in the lower limbs of young adults. The tumour shows a characteristic orderly cellular arrangement and the few mitoses belie the often rapid progress of the disease. Cytoplasmic crystals unique to this tumour may be demonstrated. The need for thorough investigation of the patient as an initial staging procedure and to provide a prognostic forecast is emphasised. Isotope scanning of the bones, brain and liver is indicated. Two cases are reported which demonstrate the value of lymphography. The nodal features found were: 1. Enlargement of a lymph node of normal internal architecture. 2. Almost complete nodal replacement by tumour. 3 . Pseudolymphomatous appearances. Follow up films are required, after an interval, to show the progress of the disease or response to therapy. A rare malignant tumour having characteristic histology, histochemistry and cellular ultrastructure was first given the descriptive title, alveolar soft part sarcoma, by Christopherson et al. in 1952. A purely descriptive name was acceptable since the cell of origin could not be determined. The histogenesis remains in doubt and the name is generally accepted. Atkinson et al. (1963) found only one alveolar soft part sarcoma amongst 122 soft tissue sarcomas of various types. Although the tumour is rare, it seems likely that some have been misdiagnosed. Smetana and Scott (1951) found the most common histological misdiagnoses were metastatic deposits of epithelial origin, usually from renal cell or hepatic cell primaries. They believed the cell of origin to be of alveolar soft part sarcoma non-chromaffin paraganglia, a view which recent developments in the study of cell ultrastructure has shown to be unlikely. Alveolar soft part sarcoma typically presents as a tender mass related to muscle in the lower limb. Of 54 cases reported in the English literature Khoo and Choo (1966) found that 32 occurred in the lower limb, seven in the upper limb and six in the retroperitoneal area. l_iebermann et al. (1966) reviewed 53 cases, of which 50 originated in the limbs. Occasional alveolar soft part sarcomas have occurred in the muscles of the neck (Smetana and Scott, 1951), scalp (Khoo and Choo, 1966), orbit (Vakil and Sirsat, 1966) and tongue (Christopherson et al., 1952; Liebermann et al., 1966; Vakil and Sirsat, 1966). Secondary deposits in the lungs (Farquharson, 1966; McGlamory and Harris, 1972) or brain (Rosenbanm et al., 1971; Ohashi et al., 1974). may be the presenting feature. Sometimes the primary tumour
may not be detected (Balfour, 1974). Non-specific symptoms such as malaise, weight loss, fatigue and fever are common. The tumour has been recorded more commonly in females and on the right side of the body. Liebermann et al. (1966) found 34 right-sided tumours in 53 cases and a female to male ratio of 2 : 1. The average age of the female patients was 20 years, at presentation, and the average male was 10 years older. A history of minor trauma preceding the discovery of a tumour mass is common. The possibility of trauma being a causal factor was discussed by Bateson (1968), who described an alveolar soft part sarcoma occurring in the thigh, adjacent to an area of myositis ossificans. Generally, any relationship with trauma is thought to be fortuitous. Survival time from the onset of symptoms or signs is very variable. Begg and Abernethy (1967) records a 15 year old girl dying within 17 weeks of discovering a swelling in the forearm. The histology gave no indication of the very malignant nature of the turnout, indeed, no mitoses were detected. On the other hand, Liebermann et al. (1966) described two patients who died of metastases 20 years after excision of the primary tumour. No cure has been recorded; all patients followed long enough have died of the disease, unless some other fatal illness intervened. Macroscopically, the tumour is usually partially encapsulated, but with invasion of surrounding muscle. The cut surface has no characteristic features, being lobulated, yellow or pink, with areas of haemorrhage or necrosis. Histology, however, reveals
Fig. l(a) - Groups of cells in an alveolar arrangement with intervening septa.
Fig. l(b) - Electron micrograph (X 40000) showing the crystalline cytoplasmic structures found in alveolar soft part Sarcoma.
Fig. 2(a) - Case 1 ; normal initial lymphograrrL Fig. 2(b) - The lymph node on the left at L5 shows enlargement, while retaining normal internal appearances. Fig. 2(c) The enlarged node is homogeneously granular with intact margins.
LYMPHOGRAPHY IN ALVEOLAR SOFT PART SARCOMA a characteristic appearance of groups of 5 - 5 0 cells surrounded by connective tissue septa containing vascular channels (Fig. la). Small collections of cells may be found protruding into vascular channels in a glomerular fashion (Smetana and Scott, 1951; Christopherson et al., 1952; Shipkey et al., 1964). Mitoses are infrequent and the cellular arrangement is very orderly, two features which belie the expected malignant course. Welsh et al. (1972) reported a comprehensive electron microscopic study of alveolar soft part sarcoma. Cytoplasmic crystals (Fig. l b ) were found which had unique characteristics separating this tumour from others to which it bears a superficial resemblance, such as non-chromaffin paraganglionomas and granular cell myoblastomas. On pathological and radiological evidence metastases have been found most commonly in the lungs
(42%), bone (19%), brain (15%) and lymph nodes (7%) (Liebermann et al., 1966). However, no thorough, systematic diagnostic search for metastases has been reported. No report of lymphography in this condition could be found in the English literature. Two further cases are reported to demonstrate the lymphographic abnormalities which have been found. CASE REPORTS Case 1. - A 22 year old male was found to have multiple round pulmonary nodules on routine chest X-ray. Open biopsy of a nodule in the lingula was performed. The histologist suggested that the lesion was a metastasis, possibly from a primary hepatoma and the patient was referred to the Royal Marsden Hospital for further investigation and management. Review of the biopsy material did not reveal the true diagnosis. Radioisotope scanning of the liver was normal. Lymphography was undertaken with the demonstration
Fig. 3(a) - Almost complete nodal replacement by tumour has occurred on the right. The left para-aortic chain shows foamy nodes. Fig. 3(b) - Considerable enlargement of left para-aortic nodes has occurred over three months.
of normal nodal appearances in the pelvis and para-aortic chains (Fig. 2a). Treatment was commenced with 5 F.U. and the patient remained asymptomatic for a year. Lymph node enlargement then occurred in the left groin and a mass became palpable in the left iliac fossa. A further lymphogram was undertaken. This showed that a node, on the left at the level of L5 vertebral body, had increased in size from 38 X 15 mm to 55 X 25 mm within the space of a year (Fig. 2b). The architecture of that node remained uniformly granular with an intact peripheral sinus (Fig. 2c). Changes of reactive hyperplasia were present in the right iUae nodes but there was no other lymphographic evidence of malignant nodal involvement. A lymph node was excised from the left groin and its histology shown to be that of alveolar soft part sarcoma. An angiographic examination was made of the pelvis and lower limbs to locate the primary turnout site, but no malignant vascularity was found. Radiotherapy was given to the pelvic and inguinal regions, followed by quadruple chemotherapy. Although some decrease occurred in size of the left iliac fossa mass, the patient's general condition gradually deteriorated and he died at home with profuse pulmonary metastases. No postmortem findings of significance are available. Case 2. - A 24 year old male presented with a painless mass, 10 cm in diameter, in the right buttock, which had been present for 6 months. There were no other symptoms. The turnout was easily excised, being encapsulated in most of its circumference, but infiltrating gluteus maximus over a small area. The histologist found the turnout to be an alveolar soft part sarcoma. The patient was transferred to the Royal Air Foxce Cade Clinic where chest X-ray revealed multiple small pulmonary metastases. Liver, brain and skeletal isotope scans were normal. Lymphog~aphy revealed grossly enlarged right iliac nodes, normal lymphoid tissue being almost completely replaced by turnout, so that only a peripheral rim was opacified by contrast medium (Fig. 3a). The nodes of the right para-aortic chain were similar but on the left the glands were foamy. 12ae left pelvic nodes appeared normal. Therapy was commenced with adriamycin, cyclophosphamide and prednisone. However, a follow-up film of the abdomen after 3 months showed enlargement of the involved nodes indicating turnout activity (Fig. 3b),
DISCUSSION Progress in the management of patients with such rare turnouts as alveolar soft part sarcoma depends u p o n reporting the thorough investigation o f those case which do occur. In this way, a knowledge of the tumour's behaviour can be accumulated. Since no case of cure has been recorded with this particular tumour, metastases must have been present at the time o f excision o f the primary mass in all cases. It may therefore be considered that all patients should receive systemic therapy and that actual demonstration o f metastases is unnecessary. Previously reported cases indicate that chest X-ray and isotope scanning of the bones, brain and liver should be carried out as part o f the patient's initial assessment, as, in this way unnecessarily radical surgery can be avoided.
At operation the primary tumour has frequently been found to be extremely vascular (Udekwu and Pulvertaft, 1965; Begg and Abernethy, 1967; Furey et al., 1969). This has also been the case with cerebral secondary deposits (Rosenbaum et aL, 1971). Tumour vascularity has been demonstrated angiographically in a primary tumour o f the thigh (Bateson, 1968) and in cerebral secondary deposits (Rosenbaum et al., 1971). Such a high p r o p o r t i o n of alveolar soft part sarcomas occur in the lower limbs, that an anglographic search for t u m o u r vascularity in that region would seem justified in those patients presenting with a metastasis due to an occult primary. Lymphography is essential in staging the extent of disease. A variety o f nodal appearances have been demonstrated. The first case described emphasises that apparently normal internal architecture does not exclude involvement by turnout. The importance of follow-up films, with injection of more contrast medium when necessary, is d e a f l y demonstrated by the eventual enlargement o f a lymph node in the drainage area of the primary tumour. The second case shows lymph node appearances more typical o f a malignant disease. The right iliac and para-aortic nodes are almost completely replaced by tumour, so that only peripheral rims o f lymphoid tissue have opacified. In the left para-aortic chains, the glands are foamy, an appearance more commonly seen in the lymphomas but occasionally found in testicular, ovarian and renal turnouts (MacDonald, 1969). Cross over of lymphatic metastases from the right side o f the pelvis to the left para-aortic group has occurred. Despite quadruple chemotherapy, the lymph nodes enlarged considerably over 3 months, indicating that a change in treatment policy was necessary. Lymphography will be found of value in cases o f this type for: 1. Correct initial staging of the disease. 2. Demonstration o f progressive tumour growth within the nodes. 3. Monitoring the response to therapy. Acknowledgements. - We are grateful to Wg-Cdr J. Chorlton, Consultant Pathologist, Royal Air Force Hospital Halton, who produced the micrographs. We are indebted to the staff of the Department of Clinical Photography, the Royal Marsden Hospital, Fulham Road, London, and Royal Air Force Hospital Halton for the illustrations. REFERENCES Atkinson, L, Garvan, J. M. & Newton, N. C. (1963). Behaviour and management of soft connective tissue sarcomas. Cancer, 16, 1552- 1562. Balfour, R. S. (1974). The alveolar soft part sarcoma. Journal o f Oral Surgery, 32, 214-220.
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