CASE REPORTS
Management of a large pleomorphic adenoma of the parotid gland
P. J. Anderson and N. R. McLean Department of Plastic Surgery, Newcastle General Hospital, U.K.
The surgical management of an extensive pleomorphic adenoma arising within the deep lobe of the parotid gland is presented. Key words: salivary gland.
Case report
A 34-year-old labourer was referred by his GP with a mass on the right side of the soft palate. This had been present for 1 year prior to presentation but he only sought medical assistance because his partner had noticed an increase in snoring. Previous medical history was otherwise unremarkable.
On examination there was no discernible facial asymmetry and no evidence of facial nerve involvement. Intra-oral examination revealed a firm mass arising on the right side of the pharynx. Computed tomography (CT) scan demonstrated a mass which arose within the deep part of the parotid gland and extended from the base of the skull down to the level of the thyroid cartilage. Open biopsy confirmed the diagnosis of pleomorphic salivary gland adenoma.
(a)
(b)
Correspondence to: Peter J. Anderson, Canniesburn Hospital, Glasgow G61 1QL, U.K.
Management consisted of surgical excision and required both a maxillary swing procedure1 combined with a mandibular split2 to allow sufficient access to allow complete excision of the tumour (Fig. 1). Excision of the tumour mass in the deep lobe was possible without involving the territory of the facial nerve.
Histological examination confirmed that this was a pleomorphic adenoma which had been completely excised. Twenty-four months later the patient is well with no evidence of recurrence and with normal facial nerve function.
This case highlights the use of a simultaneous surgical approach to both the skull base and to the pharynx, which enabled surgical
excision of an extensive pleomorphic adenoma, arising within the deep lobe, to be successfully undertaken.
References
1. Wei W, Lam KH, Sham JST. New approach to the nasopharynx: the maxillary swing approach. Head Neck 1991; 13: 200-7.
2. McGregor IA, MacDonald DG. Mandibular osteotomy in the surgical approach to the oral cavity. Head Neck 1983; 5: 457-62.
Synchronous unilateral parotid neoplasms of different histological types
S. D. Stavrianos*, N. R. McLean* and J. V. Soames*
*Head and Neck Unit, Department of Plastic & Reconstructive Surgery, Newcastle General Hospital, Newcastle upon Tyne, U.K. and ¡Department of Oral Pathology, The Dental School, Framlington Place, University of Newcastle upon Tyne, U.K.
The occurrence of multiple tumours in the salivary glands is an unusual phenomenon and the simultaneous development of tumours of different types is extremely rare. Two cases are presented with synchronous tumours of the parotid gland of different histological types. The first was a Warthin tumour in combination with a metastatic lung carcinoma and the second was a pleomorphic adenoma in combination with a non-Hodgkin’s malignant lymphoma.
Key words: synchronous parotid neoplasms; Warthin tumour; parotid metastasis; pleomorphic adenoma; malignant lymphoma.
Introduction
Salivary gland tumours account for 3% of all head and neck neoplasms.1 The vast majority of these occur in the parotid gland and 80% are benign. Of these 50-80% are pleomorphic adenomas and between 5 and 20% are Warthin tumours.1
Synchronous bilateral parotid neoplasms are uncommon2,3 and synchronous unilateral primary parotid neoplasms may be occasionally seen and are usually of one histological type.1,2 Synchronous unilateral parotid neoplasms of two separate and distinct histological types are very rare and we have identified only 33 reported cases in the English literature.1-4 In addition, metastases to the salivary glands from distant (infraclavicular) primary tumours are exceptionally uncommon.5
This paper reports two cases of synchronous unilateral parotid neoplasms of different histological types, one of which was also of distant metastatic origin. Both patients had conflicting reports on fine-needle aspiration cytology (FNAC) because of the dual pathology and the final diagnosis was only established following definitive surgical excision.
Case report 1
A 77-year-old man presented with a 6-week history of a non-painful left parotid swelling which was 4 cm in diameter and fixed to the
Correspondence to: Mr. N. R. McLean, Head and Neck Unit, Plastic & Reconstructive Surgery Department, Royal Victoria In- firmary, Queen Victoria Road, Newcastle NE1 4LP, U.K.
masseter muscle. Pre-operative investigations also revealed an ill- defined mass in the upper lobe of the left lung. FNAC of the parotid was suggestive of a Warthin tumour but atypical malignant cells consistent with a carcinoma were also reported. Following a consultation with the chest physicians, a left total parotidectomy with facial nerve reconstruction was performed.
Macroscopic examination of the excised specimen revealed a well-circumscribed mass, 2.8 x 1.5 cm in size, with a partly necrotic and haemorrhagic cut surface. Histology (Fig. 1) showed an encapsulated tumour with the characteristic features, for almost
PA
ML
half its volume, of a Warthin tumour. The remainder, including the necrotic portion, consisted of large pleomorphic undifferentiated malignant epithelial cells which also infiltrated the lymphoid stroma of the Warthin tumour. The appearances of the malignant component were consistent with a metastatic lung carcinoma.
The patient received palliative radiotherapy for the lung primary but died 3 months post-operatively.
Case report 2
A 66-year-old woman presented with a 7-month history of two non-painful left parotid swellings. Clinically there was a large 5- cm mass anteriorly and a smaller 2-cm one inferiorly to the left ear. There was no evidence of facial nerve involvement and FNAC was suggestive of a lymphoma. A left superficial parotidectomy was performed and a large tumour was found within the parotid gland.
Macroscopic examination of the excised specimen revealed two adjacent but separate tumour masses approximately 3.5 cm and 1.5 cm in diameter. The larger appeared to infiltrate surrounding tissues. Histopathological examination of this lesion showed a lymphoid neoplasm with a residual follicular architecture for most of its extent, consistent with a follicular, (becoming diffuse), non- Hodgkin’s lymphoma of centrocytic-centroblastic type. The smaller tumour was well circumscribed and comprised ductal and other epithelial elements with myxochondrial stroma, consistent with a pleomorphic adenoma (Fig. 2).
The patient received adjuvant radiotherapy and staging investigations for the lymphoma which revealed only an enlarged but asymptomatic left para-aortic lymph node. The patient is alive and disease-free 8 years post-operatively.
Discussion
Bilateral salivary gland tumours are unusual and account for 1.1% of major salivary gland lesions2,8 and for about 0.7% of tumours of the parotid gland.8 Unilateral synchronous distinct neoplasms make up less than 1% of parotid neoplasms (range 0.2-0.7%).1-3
The most common tumour to occur bilaterally (4-7.5%) or to be associated synchronously with other salivary gland neoplasms, is the Warthin tumour (papillary cystadenoma lymphomatosum or adenolymphoma,3,4,6,14 followed by the pleomorphic adenoma.6 However, bilateral synchronous tumours are reported only infrequently.8
The most commonly reported combination of synchronous parotid tumours of different histological types is that of a Warthin tumour in association with a pleomorphic adenoma (42%).4,7,8 Warthin tumour in combination with salivary tumours other than pleomorphic adenoma is less common (35%), and pleomorphic adenoma in combination with other tumours is even less so (19%).8
In addition to benign lesions, Warthin tumour has also been
reported in association with malignant tumours, either as a result of malignant transformation within the tumour itself or as a result of involvement by an independent synchronous malignancy.7-9 Squamous cell carcinoma and mucoepidermoid carcinoma arising in Warthin tumours have been documented and adenoid cystic carcinoma and accinic cell carcinoma have been also reported as independent synchronous neoplasms.7 Warthin tumour has also been occasionally reported in combination with remote unrelated malignancies of the lung, kidney, breast, bladder and thyroid gland.5,7,10
Approximately 9-14% of all parotid tumours are metastatic, usually from primary neoplasms of the head and neck.1º Metastatic disease of the parotid gland from a primary outside the head and neck area is rare, accounting for about 1.8-4% of parotid tumours, and lung, breast or kidney are the most frequently reported primary sites.10 In a clinical situation where the FNA cytology of the gland shows atypical malignant cells, in the presence of a suspected primary elsewhere, a more conservative approach than in our first case report should have been the treatment of choice.
Warthin tumours may also be associated with immune disorders, the benign lymphoepithelial lesions7 and with malignant lymphomas.11 The latter may arise either from intraparotid lymph nodes, from the lymphoid tissue that is normally found in the gland, or from the lymphoid stroma of the tumour itself.11 The association of pleomorphic adenoma and synchronous malignant lymphoma is almost certainly coincidental, and none of the 25 cases of primary malignant salivary lymphomas described by Schmid et al. showed other synchronous tumours of different histological type.12,13 In a clinical situation where there is difficulty in diagnosing separate biological lobulated masses in the gland, as in our second case report, pre-operative CT scan would nowadays settle the question.
In conclusion, although synchronous parotid tumours are uncommon, the clinician should be aware of this possibility when multifocal or multiple lesions are suspected in cases with apparently conflicting FNAC reports.
References
1. Schilling JA, Block BL, Speigal JC. Synchronous unilateral parotid neoplasms of different histological types. Head Neck 1989; 11: 179-83.
2. Turnbull AD, Frazell EL. Multiple tumours of the major salivary glands. Am J Surg 1969; 118: 787-9.
3. Janecka IP, Perzin KH, Sternschein MJ. Rare synchronous parotid tumours of different histological types. Plast Reconstr Surg 1983; 72: 789-802.
4. Krogdahl AS, Bretlau P, Hastrup N. Multiple tumours of the parotid gland. J Laryngol Otol 1983; 97: 1035-7.
5. Batsakis JG, Bautina E. Metastases to major salivary glands. Ann Otol Rhinol Laryngol 1990; 99: 501-3.
6. Curtis A, Baker NJ. Synchronous salivary gland neoplasia. Br J Oral Maxillof Surg 1997; 35: 73-4 (letter).
7. Chapnik JS. The controversies of Warthin’s tumour. Laryngoscope 1983; 93: 695-716.
8. Lefor AT, Ord RA. Multiple synchronous bilateral Warthin’s tumours of the parotid glands with pleomorphic adenoma. Oral Surg Oral Med Oral Pathol 1993; 76: 319-24.
9. Goh PMY, Cheah E. Synchronous tumours of the parotid gland with different histology. Br J Oral Maxillof Surg 1989; 27: 198-202.
10. Ravi R, Tongaonkar HB, Kulkarni JN, Kamat MR. Synchronous bilateral parotid metastases from renal cell carcinoma. A case report. Indian J Cancer 1992; 29: 40-2.
11. Shikhani AH, Shikani LT, Kuhajda FP, Allam CK. Warthin’s tumour-associated neoplasms: report of two cases and review of the literature. Ear Nose Throat J 1993; 72: 264-73.
12. Ibrahim NBM, Briggs JC. Salivary gland pleomorphic adenoma and malignant lymphoma. Histopathology 1983; 7: 445-6 (letter).
13. Schmid, U Helbron D, Lennart K. Primary malignant lymphomas localised in salivary glands. Histopathology 1982; 6: 673-85.
14. Gnepp DR, Schroeder W, Heffner, D. Synchronous tumours arising in a single major salivary gland. Cancer 1989; 63: 1219-24.
Carcinosarcoma of the submandibular salivary gland
J. Gogas, Ch. Markopoulos, V. Karydakis, G. Gogas and J. Delladetsima
2nd Propedeuticapartment of Surgery, Athens University and Medical School, Laikon Hospital, Greece
We report a rare case of submandibular salivary gland carcinosarcoma (‘true’ malignant mixed tumour) which occurred in a 77-year- old man. Microscopic examination showed a neoplasm comprised of sarcomatous elements (chondrosarcoma, rhabdomyosarcoma and osteosarcoma) with tubular salivary ductal adenocarcinoma. A short review of the literature is also presented and the poor prognosis of these tumours, in spite of complete surgical removal and additional radiation therapy and chemotherapy, is discussed.
Key words: carcinosarcoma; malignant mixed tumour; submandibular; submaxillary salivary gland.
Introduction
Carcinosarcomas are rare neoplasms composed of heterologous malignant epithelial and stromal components, which both fulfil histological criteria of malignancy.1-6 These tumours are also known as ‘true’ malignant mixed tumours. Most salivary gland tumours develop from the parotid gland and are benign; however, the incidence of malignancy in parotid gland neoplasms is 15%, in the submandibular gland 50% and in the minor salivary glands 75%.1,5-7 When a malignant component is present in a salivary gland neoplasm, it is usually derived from epithelium; stromal elements of the gland rarely give rise to primary sarcomas.1,7 Malignant transformation of both epithelial and stromal components is even less common and results in the development of a carcinosarcoma.8 In pleomorphic adenomas specifically, carcinomas may develop in 2-8%, whilst carcinosarcomas arise in less than 0.2%.8,9
We report a case of salivary gland carcinosarcoma which, because of its size, unusual malignant stromal differentiation to osteosarcoma, rhabdomyosarcoma and chondrosarcoma and its origin from the submandibular gland, is unique in the literature.
Case report
A 77-year-old man presented with a 10-year history of a hard asymptomatic lump of the right submaxillary area which, for 1 month prior to admission, had undergone rapid growth. A large, hard, fixed, wide-based mass under the mandible (on the right side) was found on clinical examination. There were no enlarged lymph nodes or distant metastases. Ultrasound of the neck showed a 10- cm (maximum diameter) solid lump with cystic elements. Scanning of the salivary glands before and after lemon tasting demonstrated the tumour origin from the right submandibular salivary gland. Computed tomography (CT) imaging revealed a 10-cm unhomogenous submandibular tumour.
The patient, under general anaesthesia underwent total resection of the tumour, together with the right submandibular salivary gland, the skin and all the infiltrated muscles. The surgical specimen measured 10×7×7cm. The cut surface showed a quite well- circumscribed mass, whitish-yellow and grey in colour, with necrotic, myxomatous hemorrhagic areas.
On paraffin section, the tumour was shown to consist of carcinomatous and sarcomatous elements, with the latter predominating. The malignant mesenchymal component was high- grade sarcoma, showing areas of chondroid, osteoid and
Correspondence to: Ch. Markopoulos, 8 Iassiou Str., 11521 Athens, Greece.
rhabdomyoid differentiation (Figs 1, 2 and 3, respectively). The malignant epithelial component was mainly tubular salivary duct carcinoma in a myxomatous hyalinized fibromatic stroma with focal squamous differentiation (Fig. 4). This carcinosarcoma had
grown from the submandibular salivary gland, which was found at the edge of the tumour.
Post-operatively, the patient received radical radiotherapy and started chemotherapy. However, 3 months later he was found to have lung metastases, shown on plain chest X-ray films and confirmed by lung CT scan.
Discussion
Carcinosarcomas are rare neoplasms, which have been described in a variety of organs including the salivary glands, lung, breast, uterus and bladder.10,11 The incidence of these ‘true’ malignant mixed tumours in the salivary glands is estimated to be 0.04-0.16% of all salivary neoplasms and 0.2-0.4% of malignant salivary tumours.1,8,10,12-14 Some sporadic cases and some small series have been described in the literature, with the largest single series of 12 cases described by Stephens et al.4 An indication of the rarity of carcinosarcomas is that to date only 45 cases have been reported recently in the English language literature,2,12,45 43 of which were reviewed by Gnepp2 in 1993. Most of these tumours arose in the parotid gland, followed in frequency by the submandibular gland, the palate2,4,7,12,13 and one in the tongue.16 The incidence was almost identical in both sexes and in every decade.1,2
Clinically, carcinosarcomas present as large masses developing from a rapidly enlarged pre-existing salivary gland tumour.2,4,5,8 In our patient the tumour showed this characteristic clinical development and maximum diameter (10 cm) is the largest reported to date.
Classification of salivary gland tumours is difficult and consequently several classification schemes have been proposed
from 1954 to 1991. Two recent classifications are the Armed Forces Institute of Pathology (AFIP) classification by Ellis et al.9 and the revised WHO classification;17,18 we used the latter.
Histologically, carcinosarcomas are biphasic neoplasms consisting of both malignant epithelial and stromal components. The epithelial components exhibit malignant glandular, squamous and undifferentiated carcinoma. The most common mesenchymal components are chondrosarcoma and fibrosarcoma. Un- differentiated sarcoma, osteosarcoma, liposarcoma and rhabdo- myosarcoma are seen very rarely.3-6,8,10 One case of carcino- sarcoma associated with a giant cell tumour has also been reported.19 Our patient showed a unique combination of chondro-, osteo- and rhabdosarcoma (Figs 1, 2 and 3, respectively).
Immunohistochemical studies show that the origin of carcinosarcoma of the salivary gland may be a common pre- cursor cell, possibly a modified myoepithelial cell.1,4-6,20,51 The analysis includes the vimentin reaction, which is usually positive for a sarcomatous component; the epithelial membrane antigen and keratin antibody reaction, usually positive for salivary duct carcinoma; and the S-100 protein and alpha-smooth muscle actin reaction, commonly negative.5,6,10,21 In our case an avidin-biotin reaction was also used to show positive reactivity of the desmin of rhabdomyoblasts (Fig. 2).
Treatment should be complete surgical removal of the tumour and proliferated tissues or lymph nodes, followed by radiation, chemotherapy or both. However, carcinosarcomas are aggressive neoplasms which in general rapidly develop metastasis or recur after resection and have a poor prognosis despite therapy. In our case, the patient developed lung metastasis 3 months after surgery while on chemotherapy.
References
1. Simpson RHW. Classification of tumours of the salivary glands. Histopathology 1994; 24: 187-91.
2. Gnepp DR. Malignant mixed tumours of the salivary glands: a review. Pathol Ann 1993; 28: 279-328.
3. Garner SL, Maves MD, Robinson RA, Barnes CH. Salivary gland carcinosarcoma: true malignant mixed tumour. Ann Otol Rhinol Laryngol 1989; 98: 611-4.
4. Stephen J, Batsakis JG, Luna MA, von der Heyden U, Byers RM. True malignant mixed tumours (carcinosarcoma) of salivary glands. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1986; 61: 597-602.
5. Henry J, Carson HJ, Tojo DP, Chow JM, Hammadeh R, Raslan WF. Carcinosarcoma of salivary glands with unusual stromal components. report of two cases and review of the literature. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1995; 79: 738-46.
6. Alvarez-Canas C, Rodilla I. True malignant mixed tumour (carcinosarcoma) of the parotid gland. Oral Pathol Oral Radiol Endod 1996; 81: 454-8.
7. Toynfon SG, Wilkins MJ, Cook HT, Stafford ND. True malignant mixed tumour of a minor salivary gland. J Laryngol Otol 1994; 108: 76-9.
8. Yamamoto Y, Kishimoto Y, Virmani A, Smith A, Vuitch F, Albores-Saavedra J, Gazdar A. Mutations associated with carcinomas arising from pleomorphic adenomas of the salivary glands. Hum Pathol 1996; 27: 782-6.
9. Ellis GL, Auclair PL, Gnepp DR. Surgical Pathology of the Salivary Glands. Philadelphia: W.B. Saunders, 1991; 129-34.
10. Gandour-Edwards RF, Donald PJ, Vogt PJ, Munn R, Min KW. Carcinosarcoma (malignant mixed tumour) of the parotid: report of a case with a pure rhabdomyosarcoma component. Head Neck 1994; 16: 379-82.
11. Gogas J. Carcinosarcoma of the breast. Acta Chir Hellenica 1974; 46: 426.
12. Bleiweiss IJ, Huvos AG, Lara J, Strong EW. Carcinosarcoma of the submandibular salivary gland. Cancer 1992; 69: 2031-5.
13. Yamashita T, Kameda N, Katayama M, Hiruta N, Nakada M, Takeda Y. True malignant mixed tumour of the submandibular gland. Acta Pathol Jpn 1990; 40: 137-42.
14. Talmi YP, Halpern M, Finkelstein Y, Gal R, Zohar Y. True malignant mixed tumour of the parotid gland. J Laryngol Otol 1990; 104: 360-1.
15. Lopez JI, Ballestin C, Garcia-Prats MD, De Augustin P. Carcinosarcoma of the parotid gland: immunohistochemical study of a case. Histopathology 1994; 25: 388-90.
16. Takata N, Nikai H, Ogawa I, Ijuhin N. Ultrastructural and immunohistochemical observations of a true malignant tumour (carcinosarcoma) of the tongue. J Oral Pathol Med 1990; 19: 261-5.
17. Selfert G, Sobin LH. Histological Typing of Salivary Gland Tumours. 2nd edn. Berlin: Springer Verlag, 1991.
18. Seifert G, Batsakis JG, Brocheriou C, et al. World Health Organization. Histological Typing of Salivary Gland Tumours. 2nd edn. Berlin: Springer Verlag, 1991: 27-8.
19. Grenko RT, Tytor M, Boeryd B. Giant-cell tumour of the salivary gland with associated carcinosarcoma. Histopathology 1993; 23: 594-5.
20. Dardick I, Hardie J, Thomas MJ, van Nostrand A WP. Ultrastructural contributions to the study of morphological differentiation in malignant mixed (pleomorphic) tumours of salivary gland. Head Neck 1989; 11: 5-21.
21. Bocklage T, Feddersen R. Unusual mesenchymal and mixed tumours of the salivary gland. An immunohistochemical and flow cytometric analysis of three cases. Arch Pathol Lab Med 1995; 119: 69-74.
Accepted for publication 20 April 1998
Biliary cystadenoma: an uncommon cause of cholestatic jaundice
P. T. den Hoed*, H. Lamerist, B. Klooswijkt and J. N. M. IJzermans*
Departments of *Surgery and ¡Radiology, University Hospital Rotterdam, The Netherlands
Introduction
Biliary cystadenoma of the liver is a rare tumour1-3 which has a strong tendency to recur after incomplete resection and has malignant potential. Differentiation of this tumour from a simple cyst of the liver is therefore important. Many techniques for the treatment of simple cysts have been described, such as marsupialization, aspiration, sclerosis or partial resection, but which cannot be performed in biliary cystadenoma.
Biliary cystadenoma of the liver mainly affects women over 40 years.4 We present the case of a young man who was admitted to the University Hospital Rotterdam with jaundice caused by a multilocular cystadenoma of the liver.
Case report
A 17-year-old boy was admitted with jaundice but no other complaints. Ultrasonography and computed tomography (CT) of the abdomen showed a 20-cm multiloculated hepatic cyst occupying the central part of the liver (Fig. 1). Endoscopic retrograde cholangiopancreaticography (ERCP) demonstrated compression of the right and left hepatic ducts; a nasobiliary drain was introduced.
Because of the central localization of the tumour, CT and 3D- imaging were performed to indicate resectability (Fig. 2). This demonstrated a cystic mass reaching from the entry of the hepatic veins into the caval vein to the displaced hilar structures. Guided by these images we decided to perform a non-anatomical resection instead of an extended left hepatectomy adjacent to the right hepatic vein.
At celiotomy, peroperative ultrasonography revealed a cystic mass in the central part of the liver, corresponding to segments IV, V and VIII. Microscopic examination of a specimen resected from the cystic wall showed histological features consistent with a hepatobiliary cystadenoma. As the mass was located in the central part of the liver, we decided to enucleate the tumour, which reduced the risk of damaging vascular or bile duct structures.
Correspondence to: P. T. den Hoed, Department of Surgery, Academic University Hospital, Rotterdam Dijkzigt, Dr Mo- lewaterplein 40, 3015 GD Rotterdam, The Netherlands.
Cystadenoma
Aorta
Right liver lobe
Portal vein
Lienal vein
Superior mesenteric vein
Inferior caval vein
Common hepatic duct
Macroscopically, resection was complete. The tumour measured about 20 cm in diameter and was found to be an elastic mass consisting of compound cysts filled with mucinous fluid.
On microscopic examination, the locules were limited by a single layer of cuboidal or columnar cells resting on a basement membrane. These cells were uniform, with round or oval nuclei, their cytoplasm was clear or faintly vacuolated and the vacuoles stained positive for mucin. In places, the epithelium formed multiple polypoid or papillary projections. The stroma supporting the epithelium was thick, compact and cellular. In conclusion, histological examination showed complete removal of a hepatobiliary cystadenoma with no malignant transformation.
The post-operative course was uneventful and the patient was discharged from hospital after 2 weeks.
Discussion
About 100 cases of hepatobiliary cystadenoma or cysta- denocarcinoma have been reported in the English literature.3,4-9 It is a relatively rare lesion, representing about 4.6% of hepatic cyst lesions.10 This tumour may be brought to clinical attention due to local effects identical to other liver masses. Compression of surrounding structures may lead to a variety of symptoms, including jaundice,4,8,11-13 abdominal pain/discomfort,4,8,11-13 ascites14 and gastrointestinal obstruction leading to nausea and vomiting.
Diagnosis of hepatobiliary cystadenoma is mainly based on abdominal ultrasonography and CT scan. Intracystic fluid, obtained by sonographically guided fine-needle aspiration, may contain elevated levels of carcinoembryonic antigen.15
CT and 3D-imaging, as indicated in this case, can be performed when resectability is in doubt. Other imaging procedures are less effective. Angiography may show that the intrahepatic arteries are displaced by the tumour; the tumour itself is avascular. Endoscopic retrograde cholangiography may be performed to exclude displacement of the intrahepatic bile ducts.
Hepatobiliary cystadenomas grow very slowly. Complications of the tumour include cholestasis caused by compression, as our patient, intracystic haemorrhage, bacterial infection, rupture and transformation into cystadenocarcinoma. Because of their pre- malignant potential4 (as well as the potential for persistence of symptomatic local disease when an incomplete excision has been carried out), hepatobiliary cystadenomas must be surgically removed completely to prevent the development of a more aggressive lesion. Therefore, biliary cystadenomas must not be confused with simple cysts, as these cysts do not require a radical resection. Simple cysts can be treated by marsupialization, aspiration and/or sclerosis, or partial resection.
As the patient described here was at risk of malignant transformation, we had to remove the cystadenoma completely despite its central localization. Trisegmentectomy would have been required if standard anatomic resection had been used. This method has been associated with a high rate of complications, including sepsis, haemorrhage, rupture and biliary and vena caval obstruction. Enucleation of the cystadenoma permitted maximal preservation of normal tissue without damaging vascular and/or biliary structures. This technique has been described by Pinson et al.16 The development of a plane of dissection immediately adjacent to the cystadenoma ensures maximal preservation of the compressed portal and venous structures serving the remaining parenchyma. Resection is facilitated for lesions located near the porta hepatis and in the interlobular region, which are otherwise difficult to manage.
Our case report supports enucleation as a successful technique in the management of hepatobilary cystadenoma, especially in cases with large, centrally located lesions. This technique should minimize the substantial mortality and early and late morbidity associated with hepatobiliary cystadenomas.
Acknowledgements
We are grateful to Dr Zondervan, Department of Pathology, University Hospital Rotterdam/Dijkzigt, for histological review of the specimens.
References
1. Benhamou JP, Menu Y. Non-parasitic cystic diseases of the liver and intrahepatic biliary tree. In Blumgart LJ (ed.) Surgery of the Liver and Biliary Tract, vol. 2. New York: Churchill Livingstone, 1985: 1015-17.
2. Ishak KG, Willis GW, Cummins SD, Bullock AA. Biliary cystadenoma and cystadenocarcinoma. Cancer 1977; 38; 322-8.
3. Moore, S, Gold PR, Lebwohl O, et al. Adenosquamous carcinoma of the liver arising in biliary cystadenocarcinoma: clinical, radiologic and pathologic features with review of the literature. J Clin Gastroenterol 1984; 6: 267-75.
4. Devaney K, Goodman ZD, Ishak KG. Hepatobiliary cystadenoma and cystadenocarcinoma: a light microscopic and immunohistochemical study of 70 patients. Am J Surg Pathol 1994; 18: 1078-91.
5. Akwari, OE, Tucker A, Seigler HF, Itani KM. Hepatobiliary cystadenoma with mesenchymal stroma. Ann Surg 1990; 211: 18-27.
6. Gourley WK, Kumar D, Bouton MS, et al. Cystadenoma and cystadenocarcinoma with mesenchymal stroma of the liver - immunohistochemical analysis. Arch Pathol Lab Med 1992; 116: 1047-50.
7. Marsh, JL, Dahms B, Longmire WP. Cystadenoma and cystadenocarcinoma of the biliary system. Arch Surg 1974; 109: 41-3.
8. Wheeler DA, Edmondson HA, Cystadenoma with mesenchymal stroma (CMS) in the liver and bile ducts. A clinicopathologic study of 17 cases, 4 with malignant change. Cancer 1985; 56: 1434 45.
9. Wolf HK, Garcia JA, Bossen EH. Oncocytic differentiation in intrahepatic biliary cystadenocarcinoma. Modern Pathol 1992; 5: 665-8.
10. Geist DC. Solitary nonparisitic cyst of the liver. Arch Surg 1955; 71: 867-80.
11. Beretta E, De Franchis R, Staudacher C, et al. Biliary cystadenoma: an uncommon cause of recurrent cholestatic jaundice. Am J Gastroenterol 1986; 81: 138-40.
12. Snedecor PA. Bile duct cystadenoma of the liver. Am J Surg 1967; 33: 581-3.
13. Van Roekel V, Marx WJ, Baskin W, Greenlaw RL. Cystadenoma of the liver. J Clin Gastroenterol 1982; 4: 167-72.
14. Forrest ME, Cho KJ, Shields JL, et al. Biliary cystadenomas: sonographic-angiographic-pathologic correlations. AJR 1980; 135: 723-7.
15. Adam YG, Nonas CJ. Hepatobiliary cystadenoma. South Med J 1995; 88: 1140-3.
16. Pinson CW, Munson JL, Rossi RL, et al. Enucleation of intrahepatic biliary cystadenomas. Surg Gynecol Obstet 1989; 168: 535-8.
Recurrent adrenocortical carcinoma in a child
B. Tršinar*, C. Oblak* and V. Smrkoljį
Divisions of *Urology and ¡Traumatology, University Medical Centre Ljubljana, Slovenia
An 8-month-old girl presented with clitoromegaly, cushingoid features and a large abdominal tumour. Ultrasonography (US) and computed tomography (CT) of the abdomen revealed a tumour of the left suprarenal gland, 12x 11 x7 cm in size. Serum levels of cortisol, testosterone and DHEA-S, and urinary extretion of 17-ketosteroids and 17-hydroxycorticoids were increased. Complete removal of the tumour was accomplished through a transabdominal approach. The diagnosis of adrenocortical carcinoma was confirmed histologically.
Three months after the first operation, a recurrent tumour of the left renal hilus, 23 x 15 mm in size, was identified by US and verified by aspiration biopsy. The tumour was removed by the transabdominal route. In this report, we discuss the diagnosis and treatment of this rare disease.
Key words: adrenal cortex; carcinoma; children.
Introduction
Adrenocortical carcinomas in children are extremely rare, they account for only 0.002% of all childhood malignancies. Most of them are functional and result in adrenal virilization or femization or Cushing’s syndrome.1 We report a case of recurrent adrenocortical carcinoma in an 8-month-old girl.
Case report
An 8-month-old girl was admitted to our Department because of clitoromegaly noticed by her mother soon after birth. Clinical examination revealed cushingoid features and a large left-sided abdominal tumour (Fig. 1).
Serum levels of testosterone, cortisol, DHEA-S and urinary 17- ketosteroids, 17-hydroxycorticoids were increased. The urinary output of adrenaline, noradrenaline, vanilmandelic acid (VMA) and homovanillic acid (HVA) was normal. Serum haematology, biochemistry and blood pressure were within the normal range.
Ultrasonography (US) and computed tomography (CT) scanning
of the abdomen revealed a tumour of 12x 11 x 7 cm, involving the left suprarenal gland. The tumour had caused caudal and medial displacement of the left kidney and lateral and cranial displacement of the spleen, while the stomach was displaced medially and anteriorly. There was no evidence of pulmonary metastases. A radionuclide bone scan disclosed no abnormalities.
Once the diagnosis had been made, the patient was operated using a transabdominal approach. A macroscopically complete removal of the tumour, weighing 565 g, was accomplished. The histological diagnosis was carcinoma of the left suprarenal gland with cellular pleomorphism, confluent necrosis, vascular invasion and atypical mitoses, yet with no extension outside the capsule. The removed regional lymph nodes were normal.
After the operation, the patient had regular follow-up examinations. During surgery and the post-operative period, the child received hydrocortisone replacement therapy. Three months after the first operation, a recurrent tumour of the left renal hilus, 23 × 15 mm in size, was discovered by US and confirmed by aspiration biopsy. The lesion was again removed by the transabdominal route. The histological pattern was the same as in the primary tumour. Since the second operation the patient has been receiving adjuvant chemotherapy with Mitotane. Six months after surgery she is doing well and has no evidence of recurrent disease.
Discussion
Only 6% of adrenal cancers in children are derived from the adrenal cortex. Females are affected more often than males (70%). As reported by some authors, these tumours most commonly occur in children between 1 and 2 years of age and during adolescence.2
Adrenocortical carcinoma in children is most frequently manifested by signs of virilization and pseudo-precocious puberty (clitoromegaly, growth acceleration, pubic and facial hair), Cushing’s syndrome, hypertension and feminization.3 An abdominal tumour is sometimes found on clinical examination, as was the case with our patient. These tumours can also be associated with congenital abnormalities, such as hemihypertrophy Beckwith-Wiedemann syndrome, and Li-Fraumeni syndrome.4
The diagnostic modalities used to define the site and the pattern of adenocortical tumour in children include US, CT scanning and magnetic resonance imaging. Inferior venacavography can be helpful in patients with a suspected neoplastic thrombus.5 Radionuclide bone studies are performed to detect bone metastases.
Roentgenograms of the hand can show advanced bone age. Our patient was evaluated by US and CT scanning.
Determinations of serum testosterone, cortisol and other adrenal steroids (DHEA-S, DHA, 17-OH-progesterone), and measurements of urinary 17-ketosteroids, free cortisol and 17-hydroxycorticoids are most often necessary for definitive diagnosis.6 In our patient, increased steroid production was demonstrated.
Complete surgical removal of the tumour, and other organs or structures if involved is the only effective treatment of adrenocortical carcinoma.7 Focal lymphadenectomy is recommended for defining the stage of the tumour.
Treatment with suppressors of steroidogenesis, such as 0,P1- DDD or Mitotane, has been indicated only in patients with recurrent or metastatic tumours.1 In children, the effectiveness of these agents has not yet been confirmed.4 Some authors recommend the use of radiotherapy. In our patient, treatment with Mitotane was instituted after the second procedure.
There is a wide diversity of opinion as to the value of different prognostic factors in children with adrenocortical carcinomas. According to some authors, the weight of the tumour is the best indicator of the degree of tumour malignancy.8 Others claim that histological parameters are more important.89 Teinturier et al.2 and Mayer et al.10 agree that complete excision of the tumour is the most important prognostic factor, even in tumours larger than 5 cm in size.
In conclusion, the described case has confirmed the high malignant potential of adrenocortical carcinoma in children, as well as its tendency to recur soon after complete surgical excision. In view of these findings, careful post-operative control of these patients is mandatory. Chemotherapy is indicated only in patients with recurrent or metastatic tumours.
References
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