66 ISABEL ET AL.

should be undertaken.'" In the case of left sided hernias, Davis describes forming a tunnel in the posterior peritoneum to bury the inferior mesenteric vein."

Paraduodenal hernias are rare causes of intestinal obstruc- tion and can cause confusion when encountered unexpect- edly in the operating room. It may be difficult to reduce the intestine if it has developed adhesions or becomes oedema- tous from prolonged incarceration. A knowledge of the anatomy is helpful in defining the problem and avoiding injury to adjacent vessels.

These hernias should be considered in the differential diagnosis of patients presenting with abdominal symptoms of obscure aetiology.

1.

2.

3.

4.

5.

REFERENCES Berardi RA. Paraduodenal hernias. Surg. Gynecol. Ohstet.

Hansmann GH, Morton SA. Intra-abdominal hernia: Report of a case and review of the literature. Arch. Surg. 1939; 39:

Meyers MA. Paraduodenal hernias: Radiologic and arterio- graphic diagnosis. Radiology 1970; 95; 29-37. Bartlett MK, Wang C, Williams WH. The surgical manage- ment of paraduodenal hernia. Ann. Surg. 1968; 168: 249-54. Mayo CW, Stalker LK, Miller JM. Intra-abdominal hernia: Review of 39 cases in which treatment was surgical. Ann. Surg. 1941; 114: 875-85.

1981; 152: 99-1 10.

973-86.

6. Freind H. Perlatzky Y. Small paraduodenal hernias. Arch.

7. Skandalakis JE. Gray SW. Surgical anatomy and embryology.

8. Parsons PB. Paraduodenal hernias. Am. J. R~entgenoI. 1953;

9. Andrews E. Duodenal hernia, a misnomer. Surg. Gytterd. Ohstet. 1923; 37: 740-50.

10. Callander CA, Rusk GY, Nemir A. Mechanisms, symptoms and treatment of hernia into the descending mesocolon: left duodenal hernia. Surg. Gynecol. Ohstet. 1935: 60: 1052-7 I .

11. Dott NM. Anomalies of intestinal rotation: Their embryologi- cal and surgical aspect. Br. J . Surg. 1923; 11: 251-85.

12. Luosto R, Ketonen P. Left paraduodenal hernia with chronic abdominal symptoms. Acta C h i . Scand. 1978; 144: 263-5.

13. Turley K. Right paraduodenal hernia: A source of chronic abdominal pain in the adult. Arch. Surx. 1979; 114: 1072-4.

14. Exner FB. Roentgen diagnosis of right paraduodenal hernia: Report of a case with survey of the literature. Am. J. R O P I ~ I ~ P I I -

15. Ghahremani GG. Meyers MA. Hernias. In: Teplick JG and Haskin ME (eds) Surgical Radiology. Vol. I . Philadelphia: WB Saunders & Co., 1981.

16. Lahy FH. Trevor W. Right paraduodenal hernia. Ann. Surg.

17. Tesson JA. Left paraduodenal hernia. Am. . I . Surg. 1957; 93:

18. Davis R. Surgery of left paraduodenal hernia. Ant. J . Surg.

SUQ. 1977; 112: 1180-3.

Surg. Clin. North Am. 1974; 54: 6, 1227-46.

69: 563-89.

01. 1933; 29: 585-99.

1945; 122: 436-43.

470-5.

1975; 129: 570-3.

Aust. N.Z. J . Surg. (1995) 65.66-68 CASE REPORT

PRIMARY FIBROSARCOMA OF THE HEART

HASAN COSKUN,* A. KURSAD BOZKURT,* G U L ~ N OZBAY,' CUYAN DEMIRKESEN;' KAMIL KAYNAK" AND FIKRET SAM] VURAL*

*Department of Thoracic and Cardiovascular Surgery and +Department of Pathology, Istanbul Uni\*er.sity Cerrahpasa Medical Faculp, Istanbul. Turkey

A case of primary fibrosarcoma of the heart, which was diagnosed in a 17 year old man, is presented. Extensive tumour resection and postoperative radiation therapy was carried out. The patient is alive after 18 months after the operation. Although these tumours are associated with very poor survival. early diagnosis and combined therapy may prolong life expectancy.

Key words: fibrosarcoma, primary heart tumours.

INTRODUCTION

Primary tumours of the heart are extremely rare. The general incidence was estimated at 0.0017% from a large autopsy series reviewed by Straus and Merlis and the majority of them were benign (75%).' Because of their rapid growth and

Correspondence: Prof. Dr Hasan Cogkun, Cerrahpasa Tip Fakiiltesi Gotiis Kalp ve Damar Cerrahisi. Anabilim Dal, Aksaray 34303, Istanbul, Tiirkiye.

Accepted for publication 3 March 1993.

systemic dissemination, they have been usually diagnosed at post-mortem. In recent years, echocardiography and com-1 puterized tomography (CT) scans have made it possible to diagnose these tumours during life with the possibility of surgical intervention. Most of that series confirm that the results are poor;2.3 however, the outlook of these patients may improve with early diagnosis and treatment.

Case report A 16 year old male was admitted with a history of precordial pain for 2 months. There was no history of previous heart

PRIMARY FIBROSARCOMA OF THE HEART 67

Fig. 1. Diagram of the tumour located in the right atrium.

disease. On admission, he was dyspnoeic at rest and rales were present at both lung bases. A chest X-ray showed a greatly enlarged heart. The CT scan with intravenous con- trast revealed a cystic tumour mass above the right atrium.

At operation, a large cystic tumour, which overlay the right side of the heart, was found when the pericardial sac was opened. About 2 L of green-black fluid was aspirated. The tumour was adherent mainly to the right atrium and extended into the pericardium. There were also nodules with a variable diameter over the inner surface of the parietal pericardium (Fig. 1). All these nodular lesions were resected, and the main tumour mass was separated by blunt and sharp dissection from the surrounding structures and a full-thick- ness portion of right atrium was removed. The defect was closed with a direct suture and macroscopically tumour excision was complete.

Microscopic examination revealed that as well as the main tumour mass, all the nodular lesions resected from the inner surface of the pericardium, including the ones that were

Fig. 2. Spindle tumour cells arranged in interlacing bundles with frequent mitoses (HE X 300).

grossly soft and yellowish, were tumour. Histologically, tumour cells possessed oval or elongated nuclei and scanty cytoplasm with indistinct borders and were arranged in bundles or fascicles. Mitoses were frequent and areas of tumour necrosis were encountered on some sections. A well established reticulum network between the tumour cells was observed by reticulum stains (Fig. 2).

After an uneventful postoperative course, the patient was discharged from the hospital 21 days postoperatively. Three weeks later he began treatment with Co 60. A tumour dose of 5000 r was administered for 30 days. Chemotherapy was not used because of the likelihood that these tumours were insensitive.

DISCUSSION Primary malignant tumours of the heart are exceedingly rare.’ Angiosarcoma is the most frequent one followed by rhabdomyosarcoma, mesothelioma, fibrosarcoma and lym- phoma, in order of f req~ency.~ Reece et al. reported only five primary malignant heart tumours in a group consisting of more than 52 500 patients who underwent cardiac opera- tions between 1961 and 1983 at the Texas Heart Institute and none of them was a fibrosarcoma.’ Molina reported a 30 year experience at the University of Minne~ota.~ There were two cases with a pathologic definition of fibrosarcoma and both of them were diagnosed at post-mortem. In a collected series during a period of approximately 25 years, Blondeau reported 533 primary tumours of the heart.3 There were 53 (10%) malignant neoplasms and 10 of them were fibrosar- coma. He pointed out that the prognosis of this tumour is worse than the others (mean survival time 0.75 years).

According to the location, some authors have classified primary heart tumours as pericardial, intracavitary and intra- mural.6 In the present case, the main bulk of the tumour was located in the right atrial myocardium but infiltrated through- out the inner surface of the pericardium as nodules and it was not possible to put this tumour in one of the categories described above.

When reviewing the literature, the survival of the patients with malignant heart tumours seldom exceeds 1 year.’v5 The case presented here documents the successful resection of

68 COSKUN E T A L .

an unusual cardiac tumour. Although the patient is well 1 '/z years after the operation, the prognosis is still uncertain. We believe that early diagnosis and a multi-disciplinary thera- peutic strategy may improve long-term survival of these patients.

3. Blondeau Ph. Primary cardiac tumors: French studies o f 533 cases. Thoruc. C c i i ~ / i n ~ ~ ~ s c . . Swgeori 1990; 38: 197-5.

4. Loffler H, Grille W. Classitication of malignant cardiac tumors with respect to oncological treatment. Thoruc. Curtlioi~risc~. Surgeoil 1990 38: 173-5.

5. Molina JE, Edwards JE. Ward HB. Primary cardiac tumors:

I .

2.

Experience at the University of Minnesota. Thorur.. Carrfio-

6. Baldelli P. Angeli DD. Dolara A, Diligenti LM. Marchi F, Salvatore L. Primary tibrosarcoma of the heart. Chest 1972;

REFERENCES I'USC. Surgeon 1990; 38: 183-9 I . Straus R, Merliss R. Primary tumor of the heart. Arch. Pathol. 1945; 39: 74-8.

Montero CG. Cardiac tumors. J . Thorut. Cardiovasc. Surg. Reece IJ, Cooley DA, Frazier OH, Hallman GL. Powers PL, 62: 234-6.

1984: 88: 439-46.

AusI. N.Z. .I. Stdrg. (1995) 65.68-70 CASE REPORT

RETROPERITONEAL MALIGNANT FIBROUS HlSTlOCUOMA CAUSING PROFUSE BLEEDING PER RECTUM

P. H. SIM,* R. PATHMANATHAN' AND R. P. JALLEH" Departments of *Surgery and 'Pathology, Unh~ersity of Malqvu. Kualu Lumpur. Muluyiu

A case is reported of retroperitoneal malignant fibrous histiocytoma (MFH) invading into the colon, causing fresh bleeding per rectum. It illustrates the difficulty encountered in the pre-operative diagnosis of this condition, especially in a patient with an atypical presentation of profuse lower gastrointestinal bleeding.

Key words: colon, malignant fibrous histiocytoma, rectal bleed, retroperitoneum.

INTRODUCTION

The retroperitoneum is the third most common site for malignant fibrous histiocytoma (MFH), after the lower and upper extremities.' Malignant fibrous histiocytoma (MFH) representing as melaena has previously been however, a review of the English literature has not revealed any cases of retroperitoneal MFH presenting with fresh bleeding per rectum. We report such a case, illustrating the difficulty in pre-operative diagnosis and with emphasis on this extremely rare cause of profuse lower gastrointestinal (GI) haemorrhage.

CASE REPORT A 65 year old man presented with a 2 month history of left hypochondria1 pain associated with anorexia, weight loss and night sweats.

On admission he was pyrexial. There was a firm and tender mass, 7 cm below the left costal margin, compatible with splenomegaly.

Correspondence: Dr R. P. Jalleh, Department of Surgery. University Hospital, University of Malaya, 59100 Kuala Lumpur. Malaysia.

Accepted for publication I7 March 1993.

Investigations showed a haemoglobin of 9.6 g/dL, white cell count of 23 400/pL with 89% neutrophils and a platelet count of 697 OOO/p,L. There was iron deficiency anaemia but serum folate, Vitamin B12, bone marrow aspirate and trephine biopsy were normal. Computed tomography (CT) suggested an enlarged spleen with multiple hypodense areas within it. which could be consistent with splenic infarct, necrosis or abscess (Fig. I ) .

After admission, he developed sudden fresh bleeding per rectum, with haemodynamic compromise, requiring trans- fusion of 8 units of blood over 48 h. Sigmoidoscopy showed fresh blood in the rectum. An angiogram was then per- formed. This showed a relatively avascular left hypochon- drial mass with no overt bleeding into the GI tract. A red blood cell scan that followed suggested bleeding into the upper GI tract. Upper GI endoscopy revealed multiple acute gastric erosions with a normal duodenum. He was started on ranitidine.

He was then haemodynamically stable but remained fe- brile with abdominal tenderness. The provisional diagnosis of splenic abscess remained. As he did not respond to intravenous Metronidazole and cefotaxime, laparotomy was performed.

A vascular left retroperitoneal tumour measuring 20 X 20 X 8cm was found invading into the splenic flexure. It had also infiltrated the posterior abdominal wall. The spleen