a child with undifferentiated sarcoma of the liver complicated with ...

Pediatric Hematology and Oncology, 21: 427–433, 2004 C Taylor & Francis Inc. Copyright ISSN: 0888-0018 print / 1521-0669 online DOI: 10.1080/08880010490457150

A CHILD WITH UNDIFFERENTIATED SARCOMA OF THE LIVER COMPLICATED WITH BRONCHOBILIARY FISTULA AND DETECTED BY HEPATOBILIARY SCINTIGRAPHY

Funda C ¸ orapçıoglu, Nazan Sarper, Hakan Demir, B. Haluk Guvenc ¸, ¨ ¨ ubir, Selami Soz Gur ¨ ¨ Akansel, and Fatma Sen Berk 2 Undifferentiated (embryonal) sarcoma of the liver (USL) is a rare malignant mesenchymal tumor principally affecting patients of pediatric age. Bronchobiliary fistula is a very rare complication in patients with liver tumor. To the authors’ knowledge, this is the first report of a bronchobiliary fistula resulting from tumor invasion in a child with liver sarcoma. A 12-year-old boy was diagnosed to have USL of the right liver lobe, invading the diaphragm. An extended right hepatectomy and total resection of the mass was performed, leaving patchy tumoral invasion of the anterior diaphragmatic surface followed by combined chemotherapy regimen. Six months after the operation, the presence of bilious sputum suggested a bronchobiliary fistula, which was confirmed by hepatobiliary scintigraphy. The patient underwent a right thoracotomy and fistula division. Although bronchobiliary fistula is a very rare complication in patients with hepatic tumors, suspicion in the appropriate clinical setting is necessary to recognize this problem. Hepatobiliary scintigraphy is the useful diagnostic procedure to define bronchobiliary fistula in children with liver tumor and clinical suspicion of bronchobiliary fistula.

Keywords. bronchobiliary fistula, hepatobiliary scintigraphy, undifferentiated liver sarcoma

Undifferentiated (embryonal) sarcoma of the liver (USL) is a rare primary malignant mesenchymal hepatic tumor that occurs in elder children [1–3]. Surgical resection remains the mainstay of treatment [4, 5]. Thoracobiliary communications in the form of either pleurobiliary or bronchocobiliary fistulas are reported complications of malignant primary or metastatic liver tumors in adulthood [6]. To our knowledge, this is the first report of a bronchobiliary fistula in a child with liver sarcoma.

CASE REPORT A 12-year-old boy was admitted to our center with a 3-month history of malaise and mild abdominal pain. On physical examination, a solid, painless abdominal mass in the upper right quadrant was found. His complete blood count, liver function test, and AFP level were within normal range. Abdominal ultrasonography, computerized tomography, and MRI findings revealed Received 2 October 2003; accepted 8 March 2004. Address correspondence to Funda Çorapçioglu, Cumhuriyet Mah. Misir Sok, EKAS yapi koop, B blok, Kat:1, Da:1, Izmit-Kocaeli, Turkey. E-mail: [email protected]

427

428

F. Çorapçioglu et al.

a large mass originating from the right liver lobe, which had a cystic appearance resembling hydatid disease. An extended right hepatectomy and total resection of the mass was performed, leaving patchy tumoral invasion of the anterior diaphragmatic surface. Histologically, the tumor was composed of diffuse, large, spindle-shaped cells in a myxoid stroma. Immunohistochemically, the tumor cells reacted with antibodies against vimentin, cytokeratin, and desmin. The pathological examination of the mass revealed USL. An adjuvant chemotherapy with ifosphamide, vincristin, and etoposide was given. The patient was lost to follow-up for 4 months following his first course of chemotherapy, and was readmitted to our center with abdominal distention and jaundice. On physical examination, a large solid mass was found in the upper right abdominal quadrant. CT and MRI scan revealed progression of the residual mass extending to the thoracic cavity but there was no lung metastasis or invasion and left hepatic lobe was normal. A second chemotherapy course with ifosphamide, carboplatin, and etoposide was given. Ten days after the chemotherapy, he presented a febrile neutropenic episode, which was successfully treated with antimicrobial therapy. On the postoperative sixth month, he was admitted to the hospital with complaints of bilious expectoration. CT and MRI scan of the thorax revealed a multiloculated right upper quadrant mass extending into the chest, with air bronchograms and air–fluid levels (Figure 1) These findings suggested the presence of a bronchobiliary communication. Hepatobiliary scintigraphy (HBS) was performed after intravenous injection of 74 MBq (2 mCi) 99m Tc N-3-bromo-2,4,6 trimethylphenylcarbamoylmethyl iminodiacetic acid (Mebrofenin), following 4 h of fasting. After perfusion images (1 s/frame for 1 min in anterior position), functional images (1 min/frame for 30 min in anterior position), and late images (3–5 min static images in anterior, lateral, and posterior positions, every 20–30 min until 4 h) were obtained with a single-head gamma camera (ADAC Argus, Epic) using a low-energy, highresolution, parallel-hole collimator in a 64 × 64 matrix at 20% energy window and 140 keV peak energy. Hepatic perfusion was normal. In functional images the left lobe of the liver was located slightly superiorly and its dimensions were abnormally increased and had irregular shape. Uptake in the right lobe couldn’t be observed because of partial lobectomy. Left lobe uptake was slightly decreased. Normal transit of radiopharmaceutical to the gut was observed at normal time. Yet, from the first hour an unusual uptake had been seen in the right inferior hemithorax. The intensity and wideness of the abnormal uptake increased by the time (Figures 2 and 3). HBS confirmed broncobiliary fistula. A right thoracotomy revealed multiple adhesions and large tumoral mass invading the diaphragm adjacent to the base of right lung. Under

A Child with Undifferentiated Sarcoma of the Liver

429

FIGURE 1 (a) Axial CT image shows air–fluid level (arrow). (b) Axial T2-weighted MRI image shows a multiloculated mass.

430


FIGURE 2 Late (120 min) images of the hepatobiliary scintigraphy of the patient. Arrowhead: left lobe of the liver; arrow: abnormal uptake in the right hemitorax.

meticulous dissection the fistula between the tumoral mass and bronchial system was excised and divided (Figure 4). The operation was terminated following tube thoracostomy. The postoperative period was uneventful.

DISCUSSION USL is a rare malignancy of elder children, which is generally considered to have a poor prognosis [4]. This tumor usually appears on CT and ultrasound as a predominantly solid mass with or without cystic areas. On the other hand, primary USL, with cystic appearance mimicking hydatid disease on imaging, is also reported [3]. The most appropriate treatment strategy has been controversial. Complete resection of the USL is the only curative therapy, although it is sensitive to chemotherapy [1, 4, 7, 8]. Recent experiences have been reported on long-term survivors with combined modality therapy, including complete surgical excision [1, 4, 7]. Because preoperative chemotherapy may allow complete resection of an unresectable tumor with clear margin, neoadjuvant chemotherapy is used routinely in the contemporary treatment of USL [1, 4]. Preoperative chemotherapy is also said


431

FIGURE 3 Late (4 h) images of the hepatobiliary scintigraphy of the patient. Arrowhead: left lobe of the liver; arrow: abnormal uptake in the right hemitorax. Note that intensity and wideness of abnormal uptake in the right hemitorax was increased while uptake of the liver was decreased.

to reduce complications during surgery [4]. In the presented case, diffuse diaphragmatic invasion may be the reason for a late bronchobiliary fistula. Although bronchobiliary fistulas are very rare complications in liver tumors, a strong suspicion in the appropriate clinical setting is necessary to recognize this problem [6]. Diagnostic imaging studies are useful to identify the communication and to delineate its location. Hepatobiliary scintigraphy with 99m Tc is a sensitive diagnostic procedure for detection of bronchobiliary fistula [9]. HBS is an established method for diagnosing a bile leak from the biliary system into various anatomical regions [10, 11]. Several reports could be found regarding biliary leakage in literature, especially posttraumatic and after liver transplantation [12, 13]. However, as far as we know, only 5 reports describe leakage into thoracic cavity, detected by HBS [14–17]. Only two of these are true bronchobiliary fistulas [9, 16]. To our knowledge this is the first report of a bronchobiliary fistula in a child with liver sarcoma detected by HBS. Surgery is the optimal therapeutic approach to bronchobiliary fistula but percutanous drainage and intravenous antimicrobial therapy may offer an alternative in patients with cancer and limited physiologic reserve,

432


FIGURE 4 Bronchobiliary fistula (arrow) thorocotomy.

especially in adults [6]. The aggressive nature of USL mandates preoperative chemotherapy since operative outcome is highly morbid in an otherwise untreated tumor. We suggest that hepatobiliary scintigraphy is a useful diagnostic technique in recognizing a hepatobiliary fistula in children with liver sarcoma.

REFERENCES 1. Bisogno G, Pilz T, Perilongo G, et al. Undifferentiated sarcoma of the liver in chilhood: a curable disease. Cancer. 2002;94:252–257. 2. Ros PR, Olmsted WW, Dachman AH, Goodman ZD, Ishak KG, Hartmen DS. Undifferentiated (embryonal) sarcoma of the liver: radiologic-pathologic correlation. Radiology. 1986;161:141–145. 3. Joshi SW, Merchant NH, Jamberk NA. Primary multilocular cystic undifferentiated (embryonal) sarcoma of the liver in childhood resembling hydatid cyst of the liver. Br J Radiol. 1997;70:314–316. 4. Kim DY, Kim KH, Jung SE, Lee SC, Park KW, Kim WK. Undifferentiated (embryonal) sarcoma of the liver: combination treatment by surgery and chemotherapy. J Pediatr Surg. 2002;37:1419–1423.


433

5. Aggarwal S, Guleria S, Dinda AK, Kumar L, Tarique S. Embryonal sarcoma of the liver mimicking a hydatid cyst in an adult. Trop Gastroenterol. 2001;22:141–142. 6. Johson MM, Chin R Jr, Haponik EF. Thoracobiliary fistula. South Med J. 1996;89:335–339. 7. Rosito P, Mancini AF, Semeraro M, et al. Malignant primary tumors of the liver in chilhood. Pediatr Med Chir. 2002;24:200–207. 8. Chuang WY, Lin JN, Hung IJ, Hsueh C. Undifferentiated sarcoma of the liver. Chang Gung Med J. 2002;25:399–404. 9. Velchik MG, Roth GM, Wegener W, Alavi A. Bronchobiliary fistula detected by cholescintigraphy. J Nucl Med. 1991;32:136–138. 10. Roca I, Ciofetta G. Hepatobiliary scintigraphy in current pediatric practice. Q J Nucl Med. 1998;42:113–118. 11. Nadel HR. Hepatobiliary scintigraphy in children. Semin Nucl Med. 1996;26:25–42. 12. Kim JS, Moon DH, Lee SG, et al. The usefulness of hepatobiliary scintigraphy in the diagnosis of complications after adult-to-adult living donor liver transplantation. Eur J Nucl Med Mol Imaging. 2002;29:473–479. 13. Colletti PM, Barakos JA, Ralls PW, Siegel ME, Halls JM. Hepatobiliary scintigraphy and scintiangiography in abdominal trauma. Clin Nucl Med. 1987;12:901–909. 14. Lee JK. Tc-99m DISIDA hepatobiliary scintigraphy showing bile leakage into the thoracic cavity. Clin Nucl Med. 2001;26:861–862. 15. Suehiro M, Ishimura J, Fukuchi M. Detection of bile leakage into the thoracic cavity by hepatobiliary scintigraphy. Ann Nucl Med. 1991;5:167–169. 16. Rubin RH, Livni E, Babich J, et al. Pharmacokinetics of fleroxacin as studied by positron emission tomography and [18F]fleroxacin. Am J Med. 1993;94:31–37. 17. Marlatt SW, Caride VJ, Prokop EK. Incidental demonstration of pericardial fistula during hepatobiliary scintigraphy. J Nucl Med. 1991;32:518–522.