Malignant Histiocytoma of the Rectum: Report o f a Case*

PRAMEELA VERMA,

M.S., UMA CHANDRA, M.D., PARAMJEET SINGH BHATIA, M . D .

ALTHOUGH TUMORS OF HISTIOCYTIC ORIGIN h a v e

been reported previously, their malignant potential has been described only in a 1964 report by O'Brien and Stout? a Recently, Soule and Enriquez a5 classified four histologic types of such tumors, which microscopically had a bizarre and often malignant appearance. These less common malignant minors vary in appearance, ranging from predoininantly fibrogenic to a pronounced epithelioid or histiocytic appearance. Soule and Enriquez reported that these tumors showed a transition from atypical to malignant. The four varieties classified were atypical fibrous histiocytonla, malignant fibrous histiocytoma, malignant histiocytoma, and epithelioid sarcoma. Although these tumors are usually located on the skin and in subcutaneous tissue, they have been reported to occur in the bronchus, t hmg," inguinal canal, a joints, 4 scrotum, T M kidney, 7't~ tendon sheath, ~ urinary bladder, TM and vagina, t~ A case of inalignant abdominal histiocytoma in an 8-year-old boy was reported by Musiej-Nowakowska et a l . v'- This is the only reported case of malignant histiocytoma of the gastrointestinal tract. We present what we believe to be the first report of a malignant rectal histiocytoma. R e p o r t o f a Case A 3S-year-old man was admitted to the All India Institute of Medical Sciences in May 1977 with complaints of loose bowel movements with mucus, loss of weight, and a two-year history of abdominal pain. He had ten to 15 bowel movements a day. Stools were semisolid, with an offensive odor, and were accompanied by large amounts of mucus. The abdominal pain was described bv the patient as dull aching in the umbilical region; the pain did not radiate and was not related to food intake. The patient had occasionally' experienced severe, colicky pain in the umbilical region. associated with an inability to pass flatus, but no distention. Micturition was normal. Although his appetite was unimpaired, the patient had lost a considerable amount of weight. He was not an alcoholic and did not smoke. * Received for publication September 11, 1978. Address reprint requests to Ms. Verma: Department of Surgery, Teaching Block, 5th Floor, Room 5025, All India Institute of Medical Sciences, New Delhi-16, India.

From the Departments of Surgery, Radiolo~, and Pathology, All India Institute of Medical Sciences, New Delhi, India Physical examination revealed that the patient was poorly nourished and anemic. T h e liver was slightly enlarged. Rectal examination revealed an irregular mass, which bled when touched. Results of laboratory tests showed hemoglobin, 8 g/dl; leukocyte count, l l,300/bd; erythrocyte sedimentation rate, 40 mm/hour: packed cell volume, 25 per cent. Results of liver function tests, blood urea, blood glucose, and electrolytes were within normal ranges. Roentgenograms of the chest did not reveal an)' abnormality. Proctosigmoidoscopic and barium-enema examinations (Fig. 1) revealed a polypoid friable growth, involving 10-12 cm of the 1-ecturn and distorting the normal mucosa. The growth arose mainly from the posterior wall and extended outward, increasing the presacral space. T h e rest of the colon was normal. A biopsy specimen of the growth was reported to be malignant, and compauble with a diagnosis of malignant histiocytoma. Exploratory laparotomy revealed that the tumor filled most of the pelvis displacing the bladder upwards. The pelvic peritoneum was thickened. The lymph nodes near the origin of the inferior mesenteric vessels were enlarged and necrotic. T h e left ureter was dilated. T h e r e was no evidence of metastasis in the liver, and the rest of the abdominal organs were normal. An abdominoperineal resection of the rectum was performed. The necrotic lymph nodes were removed along with the tumor and a left iliac end-colostomy was created. T h e perineal wound was closed in layers. The immediate postoperative course was complicated by a burst abdomen and some difficulty with micturition. These complications were managed successftdly and the patient was discharged two months after operation. He had not been treated with either chemotherapy or irradiation.

The resected specimen (Fig. 9) measured -99 cm, and consisted of part of the sigmoid colon, rectum, and anal canal. On the serosal aspect, there were n u m e r o u s l o b u l a t e d m a s s e s e a c h 1-_9 c m i n d i a m e t e r , containing hemorrhagic, necrotic material. On the mucosal aspect, there was a large, ulcerated, fungati n g g r o w t h , 1-9 c m i n d i a m e t e r , l o c a t e d a b o u t 6 c m proximal to the anal verge. There were a few satellite n o d u l e s , e a c h 1 c m i n d i a m e t e r , a r o u n d t h e m a i n le.. sion containing hemorrhagic, necrotic material. The section of colon proximal to the growth was dilated. The mucosa between the ulcerated growth and anal verge was flattened. Microscopic examination of the lesion revealed that the tumor extended from the lamina propria to the

0012-3706/79/0400/0179/$00.70 9 American Society of Colon and Rectal Surgeons

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Fro. 1. Left, Bariumenema study, showing lobulated mass starting 3 cm from the anal verge and extending 10-12 cm. Right, barium-enema study (lateral view), showing marked increase in the presacral space.

serosa. T h e surface epithelium was partially ulcerated and, in some areas, the epithelium had t h i n n e d out and stretched o v e r the t u m o r . T h e cancerous cells had infiltrated the lamina p r o p r i a (Fig. 3), d e s t r o y i n g the muscularis mucosae; in the s u b m u c o s a a n d muscularis propria, w h e r e they had infiltrated the area b e t w e e n the muscle fibers, which had b e e n f r a g m e n t e d a n d s e p a r a t e d f r o m each other. T h e t u m o r cells were also seen on the serosal aspect o f the intestine. T h e t u m o r consisted of p l e o m o r p h i c a n d bizarre

ceils with large n u m b e r s o f T o u t o n a n d t u m o r giant cells (Fig. 4). T h e cells h a d a b u n d a n t f o a m y cytoplasm (Fig. 5) with large vesicular h y p e r c h r o r n a t i c nuclei. A few o f these giant cells showed active phagocytosis o f lymphocytes, n e u t r o p h i l s a n d erythrocytes. S o m e o f the neoplastic cells h a d b e c o m e spindle-shaped. T h e t u m o r cells were f o u n d to contain sudanophilic m a t e rial in their cytoplasm. T h e resected m a r g i n s were free o f t u m o r . A diagnosis o f m a l i g n a n t histiocytoma was m a d e .

Fla. 2. Resected specimen, showing the large ulcerated fungating gTowth along with satellite nodules in the rectum.

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Fro. 3. P h o t o m i c r o g r a p h , s h o w i n g histiocytic t u m o r infihration o f the lamina propria of" the rectal wall (hematoxylin a n d eosin: x 30).

Fro. 4. P h o t o m i c r o g r a p h o f t u m o r d e s t r o y i n g the muscularis m u c o s a e a n d infiltrating the s u b m u c o s a . Note n u m e r o u s t u m o r giant cells m i x e d with histiocytes. (hematoxylin a n d eosin; x 200).

Discussion

Musiej-Nowakowska et a l . , t'2 the patient was an 8-year-old boy. Metastases of the tumor to lymph nodes and lungs were described by Wasserman and Stuard tr and Soule and Enriquez. ta More recently, local recurrence of the tumor after local excision was reported by Hughes et al., 6 and Wasserman and Stuard. lr These investigators

Fibrohistiocytic tumors are a heterogeneous group. Neoplastic activities vary from benign to malignant. O'Brien and Stout la were the first to describe the malignant potential of these tumors. They defined the tumors as malignant only when they metastasized. More recently, Kempson and Kyriakos 8 and Soule and Enriquez is have described the specific histologic criteria for malignant fibrous histiocytoma. Kempson and Kyriakos 8 have described the characteristics of malignancy as a pattern of histiocytes and bizarre histiocytic giant cells intermingled in a fibrous stroma; the fibroblasts are arranged in a storiform pattern, and there are foam cells and abnormal mitosis. Soule and Enriquez x5 stated that, histologically, the malignant histiocytoma had a more monomorphic pattern than did the malignant fibrous histiocytoma. There are several theories regarding the etiology of the tumors. Kauffman and Stout r suggested that they arise from tissue histiocytes. Kempson and McGavran u suggested that this type of tumor develops during the reparative process after tissue damage. The average age of patients with malignant histiocytoma, as reported by Soule and Enriquez, is was 50 years. Our patient was 38 years old. In the case of malignant histiocytoma of the stomach reported by

FIG. 5. P h o t o m i c r o g r a p h o f a n o t h e r area o f the t u m o r , showing x a n t h o m a t o u s c h a n g e s (hematoxylin a n d eosin; x 200).

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s u g g e s t e d that local r e c u r r e n c e is m o r e c o m m o n t h a n d i s t a n t metastasis, T r e a t m e n t o f the t u m o r t h r o u g h wide local excision has b e e n a d v o c a t e d by K a u f f m a n a n d S t o u t / O ' B r i e n a n d Stout, ~a a n d W a s s e r m a n a n d S t u a r d Y H o w e v e r , S o u l e a n d E n r i q u e z ~5 f o u n d , t h a t in five p a t i e n t s with m a l i g n a n t h i s t i o c y t o m a , local r e c u r r e n c e a f t e r e x c i s i o n o c c u r r e d 1 1 times; this h i g h r a t e o f r e c u r r e n c e has b e e n r e p o r t e d by o t h e r i n v e s t i g a t o r s , a n d t h e r e f o r e , r a d i c a l r a t h e r t h a n local excisio.n is r e c o m m e n d e d . T h e five-year s u r v i v a l rate o f p a t i e n t s with m a l i g n a n t h i s t i o c y t o m a i n t h e series o f S o u l e a n d E n r i q u e z ~ was 50 p e r c e n t a n d the t e n - y e a r s u r v i v a l rate was 0. D u r i n g t h e 1 4 - m o n t h f o l l o w - u p p e r i o d , o u r p a t i e n t has r e m a i n e d well.

Summary A case o f m a l i g n a n t h i s t i o c y t o m a o f the r e c t u m has b e e n d e s c r i b e d . We believe this is the first r e p o r t o f a case o f this type. O t h e r r e l e v a n t r e p o r t s are r e v i e w e d .

References 1. Bates T, Hull OH: Histiocytoma of the bronchus: Report of a case in six-year-old child. AmJ Dis Child 95: 53, 1958 2. Brunn H: Two interesting benign hmg tumors of contradictory histopathology: Remarks on the necessity for maintaining the chest tumor registry. J Thorac Surg 9:119, 1939

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3. Cole AT, Straus FH, Gill WB: Malignant fibrous histiocytoma: An unusual inguinal tumor. J Urol 107:1005, 1972 4. De Santo DA, Wilson PD: Xanthomatous tumors of joints. J Bone Joint Surg (Am) 21: 531, 1939 5. Garrett CA: Tumors of the xanthoma type. Arch Surg 8: 890, 1924 6. Hughes JP, M'ills NL, Lynch RC, et al: Malignant fibrous hisnocytoma. South MedJ 68: 1219, 1975 7. Kauffman SL, Stout AP: Histiocytic tumors (fibrous xanthoma and histiocytoma) in children. Cancer 14: 469, 1961 8. Kempson RL, Kyriakos M: Fibroxanthosarcoma of the soft tissues: A type of malignant fibrous histiocytoma. Cancer 29: 961, 1972 9. Kempson RL, McGavran MH: Atypical fibroxanthomas of the skin. Cancer 17: 1463, 1964 10. Klugo RC, Farah RN, Cerny JC: Renal malignant histiocytoma. J Urol 112: 727, 1974 1 I. Meares EM Jr, Kempson RL: Fibrous histiocytoma of the scrotum in an infant. J Urol 110: 130, 1973 12. Musiej-Nowakowska E, Woszczyk W, Gizycka I, et al: Histiocytoma malignum ventriculi u 8-1emeigo chlopca. Pediatr Pol 48: 85, 1973 13. O'Brien JE, Stout AP: Malignant fibrous xanthomas. Cancer 17: 1445, 1964 14. Rosas-Uribe A, Ring AM, Rappaport H: IMetastasizing retroperitoneal fibroxanthoma (malignant fibroxanthoma). Cancer 26:827, 1970 15. Soule EH, Enriquez P: Atypical fibrous histiocytoma, malignant fibrous histiocytoma, malignant histiocytoma, and epithelioid sarcoma; a comparative study of 65 tumors. Cancer 30: 128, 1972 16. Stearns MM, Mitchell AD, Powell NE, et al: Fibrous histiocytoma of the bladder. J Urol 115:114, 1976 17. Wasserman TH, Stuard ID: Malignant fibrous histiocytoma with widespread metastases: Autopsy study. Cancer 33: 141, 1974 18. Webb MJ, Symmonds RE, Weiland LH: Malignant fibrous histiocytoma of the vagina. Am J Obstet Gynecol 119: 190, 1974

Memoir PINKUS, Louis R., New Hyde Park, New York; born February 14, 1916, New York, New York; University of Lausanne Medical School, 1941; internship Bronx Hospital, New York City; served with the United States Armed Forces in World War II. Dr. Pinkus was an Affiliate Member of the American Society of Colon and Rectal Surgeons, having been elected to membership in 1971. He was a Diplomate of the American Board of Surgery, a Fellow of the American College of Surgeons, and a m e m b e r of the American Medical Association, New York State Medical Society, Queens County Medical Society, and New York Society of Colon and Rectal Surgeons. He was on the staff of Long Island Jewish and North Shore Hospitals. Dr. Pinkus died December 16, 1978.

Malignant histiocytoma of the rectum: report of a case.

Malignant Histiocytoma of the Rectum: Report o f a Case* PRAMEELA VERMA, M.S., UMA CHANDRA, M.D., PARAMJEET SINGH BHATIA, M . D . ALTHOUGH TUMORS O...
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