PSEUDONEOPLASTIC FORM OF SCHISTOSOMIASIS MANSONI

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Gertrude Madison Kelley
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1 GASTROENTEROLOGY Copyright 1968 by The Williams & Wilkins Co. Vol. 54, No.1 Printed in U.S.A. PSEUDONEOPLASTIC FORM OF SCHISTOSOMIASIS MANSONI SERGIO A. BICALHO, M.D. Department of Clinical Medicine, Medical Faculty of the Minas Gerais Federal University, Belo Horizonte, Brazil Among the chronic forms of schistosomiasis mansoni, a rare one has been described which is characterized by the development of a pseudotumor. The locus for its development is the submucous or subserous tissue of the intestinal wall, and it represents an anomalous response to the eggs of the parasite. An extremely intensive and rapid hyperplastic formation of connective tissue takes place around the granulomata produced by the eggs. More rarely, this tumorlike growth is formed by a localized accumulation of egg-generated granulomata without deviation from the normal fibrotic reaction. The purpose of this report is to describe a case of this form of schistosomiasis mansoni, in which the pseudotumor grew from the rectum into the retroperitoneal space and was associated with alteration of bowel movement and micturition. Case Report The patient, first seen by us on September 5, 1966, was a 55-year-old married white male, born in Itabirito (Minas Gerais, Brazil) and living in Belo Horizonte (Minas Gerais). His past history was noncontributory. He had never suffered from significant infections or from tumors. He was a peasant, laboring 5 hr daily in a water-logged rice plantation. He had never experienced pruritus of the legs or dermatitis after work. One year previous to our first examination he had complained of increasing tiredness, weakness, and anorexia, yet he had continued working. In the beginning he had not lost weight, nor had he had fever, anemia, mucous Received July 17, Accepted August 28, Address requests for reprints to: Dr. Sergio A. Bicalho, Department of Clinical Medicine, Medical Faculty of the Minas Gerais Federal University, Rua Sao Paulo, 2295, Bela Horizonte, Brazil. 105 or bloody stools, or any other alteration of bowel function. However, 3 months later, he developed diarrhea, with up to six watery brown stools a day, and edema of the legs. He was referred by his physician for parasitological stool examination which was reported to be positive for eggs of Schistosoma mansoni and Necator americanus, for larvae of Strongyloides stercoralis, and for cysts of Entamoeba histolytica. Specific therapy was instituted with tetrachloroethylene, one dose of 5 ml by mouth, repeated after 7 days; thiabendazole, one dose of 50 mg per kg body wt, repeated after 7 days; and chlorophenoxamide (400 mg) associated with iodochlorhydroxyquin (500 mg) three times a day each, for 10 days. Under this treatment the parasites disappeared from the stool with exception of the eggs of S. mansoni for which treatment was refused by the patient. However, his general condition did not impreve. One month later the patient felt a small tumor just below the umbilicus which grew very quickly. It was a round, hard, nontender mass. When the patient bent forward, pulsations of the mass could be felt. Seven months later the tumor was reported to be the size of a football. The patient could no longer walk properly, his legs were emaciated, and he had severe, watery diarrhea. Because of this intensification of the symptoms he went to see a physician who suggested that the large tumor might be a sarcomatous neoplasm. On September 10, 1966, the patient was hospitalized for surgical exploration. On admission his main complaints were presence of a large abdominal tumor, emaciation of the legs due to venous compression, watery diarrhea, and pollakiuria (15 to 20 micturitions a day). Physical examination revealed a still well nourished man, 172 cm tall and weighing 65 kg. The temperature was 36.3 C; the pulse was 84 with normal rhythm; respirations were 16 per min; the blood pressure was 120/80. Heart and lungs were normal, no murmurs were heard. The abdomen was distended and not tender. A tumor was found to extend upward from the suprapubic region until 7 cm above the umbilicus and to expand

106 CASE REPORTS Vol. 54, No.1 to both sides. Its surface was smooth and the consistency hard; it was sharply limited. Liver and spleen were not palpable; there were no ascites.

Laboratory findings,' Hb, 16 g per 100 ml; hematocrit, 45%; red blood count, 5.2 million mm"; white count, 8,450 per nun".

2 106 CASE REPORTS Vol. 54, No.1 to both sides. Its surface was smooth and the consistency hard; it was sharply limited. Liver and spleen were not palpable; there were no ascites. The peripheral lymph nodes were not enlarged. The legs presented extensive pitting edema; their veins were slightly dilated, especially the left. Laboratory findings,' Hb, 16 g per 100 ml; hematocrit, 45%; red blood count, 5.2 million mm"; white count, 8,450 per nun". Differential count, polymorphonuclears, 66%; stabs, 1%; eosinophils, 5%; lymphocytes, 26%; and monocytes, 2%; sedimentation rate, 16 mm in 1 hr; coagulation time, 9 min (Lee-White); bleeding time, 2 min (Duke); prothrombin activity, 84% (Quick); total serum proteins, 7.6 g per 100 ml; albumin, 3.2 g per 100 ml; globulin, 4.4 g per 100 ml. Blood serum electrophoresis: albumin, 3.2 g per 100 ml (42.2%); globulins: ( " 0.4 g per 100 ml (5.5%); ( 2, 0.8 g per 100 ml (10.2%); (3,1.1 g per 100 ml (14.6%); y, 2.1 g per 100 ml (27.5%). Serum bilirubin, alkaline phosphatase, thymol turbidity, and thymol flocculation were normal. Blood urea: 30 mg per 100 ml. The feces contained eggs of Schistosoma mansoni, but no other parasites. A percutaneous liver biopsy was essentially normal. It did not contain any schistosomotic granulomata, nor was there any periportal fibrosis. Proctosigmoidoscopic examination showed minimal alterations of the mucosa, redness, and adherent mucus. Five biopsy pieces were taken from the valves, and in these 40 immature viable forms of S. mansoni eggs were found (second and third stage of development) as well as granulomata and calcified eggs. Proctitis due to Schistosoma was found in another biopsy specimen. Radiologic examinations: Chest X-ray was normal. A survey film of the abdomen showed a homogenous well limited mass in the inferior part of the abdomen. A barium enema revealed a dolichocolon and an extrinsic compression of the lower portion of the descending colon (fig. 1). On September 17 a laparotomy was performed. The serosa as well as the surface of the liver were normal. A large mass growing retroperitoneally had dislocated the small bowel forward and the colon laterally. A fibrous smooth capsule seemed to have protected it from undergoing adhesions. It was pedunculated, its small basis being located at the posterior wall of the rectum. The slender pedicle originating there was 5 cm long; from there the large tumor expanded into the retroperitoneal space. The dissection was not difficult. It was possible to mo- FIG. 1. Barium enema showing the extrinsic compression of the colon.

It consisted chiefly of proliferating granulomatous tissue in which foci of inflammation with epithelioid cells, surrounded by eosinophils, lymphoplasmocytes, and fibrocytes, were found.

3 January 1968 CASE REPORTS 107 FIG. 2. The hard tumoral mass cut sagitally. bilize it down to its base, where it was readily cut off (fig. 2). Macroscopic examination revealed an encapsulated hard mass, with a short pedicle; it weighed 1.8 kg. Microscopic examination showed its subserous origin. It consisted chiefly of proliferating granulomatous tissue in which foci of inflammation with epithelioid cells, surrounded by eosinophils, lymphoplasmocytes, and fibrocytes, were found. This granulomatous tissue had a moderately developed vascular network and collagen fibers without hyaline degeneration. Many schistosomal granulomata were seen,,most of them having a fibrotic structure (fig. 3). The postoperative course was uneventful. One month later the patient was treated for schistosomiasis with nitrothyazole (1- (5-nitro-2 thyazolyl) -2 imidazolidinone) (Niridazole in the United States). The dosage used was 500 mg three times a day for 7 days and phenobarbital, 100 mg twice a day, as a preventive for possible neuropsychiatric complications. A proctoscopic examination performed 9 months after the treatment revealed a normal mucosa, and nine rectal biopsy fragments showed only calcified eggs and shells; no viable forms of S. mansoni eggs were found. He is now in excellent health. Discussion Hyperplastic pseudoneoplastic forms of Schistosoma mansoni have been rarely reported.1-7 However, in endemic areas it is essential to be aware of this form, because it may simulate true neoplasms.! There are other forms of schistosomiasis which may simulate tumors, e.g., the stenosing form1o or the polypoid form.h The case here presented is different from those chronic fibrotic intestinal forms which cause stenosis of the sigmoid or of the rectum and in which generally no excessive tissue reaction is found. It differs also from the adenomatous polyps, in which excessive growth is restricted to the intestinal mucosa. The factor responsible for the typical lesions in schistosomiasis is the eggs which have not been eliminated with the stool. When the eggs are carried into ectopic sites they usually effect an inflammatory granulomatous reaction with a tendency to heal by fibrosis and resorption. The worms also stimulate the formation of new connective tissue. Probably according to the

108 CASE REPORTS Vol. lj,~, No.1 FIG. 3. Schistosomal granulomata surrounded by proliferating granulomatous tissue composed of reticuloendothelial cells and collagen fibers. X 60.

2 In the first instance there would be no particular tissue reaction, but simply a marked calcification around the eggs.

4 108 CASE REPORTS Vol. lj,~, No.1 FIG. 3. Schistosomal granulomata surrounded by proliferating granulomatous tissue composed of reticuloendothelial cells and collagen fibers. X 60. host's immunological response, two types of reaction, hypoergy and hyperergy, may be noted.2 In the first instance there would be no particular tissue reaction, but simply a marked calcification around the eggs. In the latter, even if the number of eggs is small, an explosive hyperplastic reaction will occur. No explanation can be offered for the rarity of this form. Pseudotumor development has been found to be associated with the hepatosplenic form of schistosomiasis mansoni. In the present case the intestinal form was present, the liver biopsy being essentially normal. Summary A case of the pseudoneoplastic form of Schistosoma mansoni is presented with the development of a huge hyperplastic mass growing into the retroperitoneal space, caused by S. mansoni eggs in an ectopic site, the subserous retrorectal tissue. The rapid evolution suggested malignancy despite the good general state of the patient. The correct diagnosis was made at laparotomy. REFERENCES 1. Bicalho, S. A Sabre as neoformal(oes conjuntivo-hiperplasticas pseudo-tumorais, na esquistossomose mansoni. Gen. 19: Bicalho, S. A., and R. P. Souza Polipo esquistossom6tico do intestino delgado. 0 Hospital 66: Bogliolo, L Patologia da Esquistossomose mansoni. Rev. Bras. Malariol. 11: Carreno, M. P., and A. Diez Las neoformaciones bilharzianas del intestino grueso (Los Bilharziomas). Gen. 10: Koppisch, E Estudios sobre la esquistosomiasis de Manson en Puerto Rico. VI Anatomia Patologica observada en la poblaci6n puertoriquena. Puerto Rico J. Pub. Health Trop. Med. 16: Mohammed, A. S Contribution to the study of Egyptian schistosomiasis. Ann. Trap. Med. Parasit. 24: Sobrinho, J., and F. O. Kelsch Aspectos tumoras da esquistossomose do colon. Rev. Brasil. Radiol. 2: Stephens, R. R Bilharziasis as a differ-

5 January 1968 CASE REPORTS 109 ential diagnosis for abdominal tumours. Central Afr. J. Med. 12: Stock, F. E Granulomas of the large bowel simulating malignant disease. Brit. J. Surg. 51: Jaffe, R Lesiones intestinales produzidas por la bilharzia mansoni encontradas en Venezuela. Rev. Po lie!. Caracas 47: Alvarenga, R. J., and E. P. Pinto Polipos esquistossom6ticos do reto. Rev. Ass. Med. Minas Gerais 11: 8-12.

SCHISTOSOMIASIS (BILHARZIA)

TREMATODES SCHISTOSOMIASIS (BILHARZIA) Hazem Al-Khafaji LECTURE-12- HISTORICAL FACTS... First described by German pathologist Theodore Maximilian Bilharz Bilharz performed autopsies on Egyptian patients