å¡ CASE REPORT å¡ Mesenchymal Hamartoma of the Liver: Report of an Adult Case and Review of the Literature Masaaki Wada, Eiji Ohashi, Hirofumi Jin, Masashi Nishikawa, Shigeyuki Shintani, Masato Yamashita, Masakatsu Kano, Naoki Yamanaka*, Takashi Nishigami** and Takashi Shimoyama A case of mesenchymal hamartoma of the liver incidentally found to be a hyperechoic mass by abdominal ultrasonography in a 62-year-old male is described. Computed tomography and magnetic resonance imaging of the liver confirmed the presence of a well-demarcated inhomogeneous mass in the left medial segment. Hepatic arteriography revealed the mass to be generally hypovascular. Histopathologic diagnosis of the resected specimen was a solid form of benign mesenchymal hamartoma, a rather rare lesion for the patient's age. Fifteen adult cases of hepatic mesenchymal hamartoma in the literature including the present case are reviewed. (Internal Medicine 31: 1370-1375, 1992) Key words: ultrasonography, computed tomography, magnetic resonance imaging, angiography

Introduction The term mesenchymal hamartoma has been widely accepted since its introduction in 1956 by Edmondson (1) who stated that various cystic lesions of the liver are all variants of the same condition. It is a rare benign lesion which occurs predominantly in children (2). Only 14 cases are known to have been diagnosed in patients over the age of 19 years (3-16). In this report, we present a case of this uncommon tumor found in an older male and a review of the literature. Case Report A 62-year-old Japanese male was referred to our hos pital on August 31, 1990 for evaluational investigation of a liver tumor which was incidentally discovered by abdominal ultrasonography (US) during a periodic health check-up at a private hospital. The patient had a 12-year history of diabetes mellitus treated with insulin. The past history was also positive for renal stone at the age of 57. On the day of admission, he was completely Physical examinations of the neck, thorax, abdomen, asymptomatic. and extremities exhibited no remarkable findings. Lab oratory data showed no abnormalities in blood chemistry,

peripheral blood cell count, and tumor markers including ar-fetoprotein, carcinoembryonic antigen, and protein induced by vitamin K absence II except for an elevated level of fasting blood glucose (187 mg/dl). Abdominal US revealed a well-demarcated, 6 x 5 cm, hyperechoic mass containing a cluster of small anechoic structures in the medial segment of the left lobe of the liver (Fig. 1). Precontrast computed tomography (CT) demonstrated the presence of an inhomogeneous, low density, hepatic mass arising from the left medial seg ment. Dynamic CT showed no early enhancement of a mass. On the delayed scans, however, a slightly enhanced soft tissue density area was observed to be mixed with the low-density area that was not enhanced (Fig. 2). The No swelling of intraperitoneal lymph could be other intrahepatic segments were freenodes of mass lesions. seen on CT scans. Magnetic resonance images were obtained in the axial plane using a multislice spin echo technique. Tl-weighted images showed a well circumscribed, inhomogeneous, low-intensity mass in the left lobe of the liver (Fig. 3a). The high-intensity spot in the mass wasimages considered to possibly hemorrhage. T2-weighted revealed that theindicate mass was inhomo geneous inside, presenting a markedly high signal inten sity (Fig. 3b). In the arterial phase of the common hepatic arteriography (Fig. 4a), a generally hypovascular

From the Fourth Department of Internal Medicine, *the First Department of Surgery and **the Second Department of Pathology, Hyogo College of Medicine, Nishinomiya Received for publication May 15, 1992; Accepted for publication October 12, 1992 Reprint requests should be addressed to Dr. Masaaki Wada, the Fourth Department of Internal Medicine, Hyogo College of Medicine, 1 Mukogawa-cho, Nishinomiya 663, Japan 1370

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Mesenchymal Hamartoma of the Liver

Fig. 1. Abdominal US shows a hyperechoic mass in the left medial segment of the liver. Small anechoic structures are seen gathered together in the mass.

Fig. 3. a) Magnetic resonance imaging of the liver shows a well circumscribed mass with a low signal intensity in the left lobe on the Tl-weighted image (SE 500/22). The high-intensity spot seen in the mass might be indicative of hemorrhage, b) The T2-weighted image (SE 2000/120) of the liver reveals an inhomogeneous mass with an increased signal intensity.

needle biopsy of the lesion was performed, but the sample was not diagnostic. Because these examinations failed to completely rule out malignant tumors, left medial segmentectomy of the liver and cholecystectomy Fig. 2. Delayed scan of dynamic CT shows an inhomogeneous, were carried out on December 25. At surgery, the mass was dark red and protruding low-density mass in the left hepatic lobe. A part of the mass is slightly from the left hepatic lobe. The resected tumor was enhanced. elastic, firm, 6x6x 4.5cm in size, well demarcated from the surrounding liver parenchyma, and not en mass was observed in the region extending from the left medial segment to the right anterior segment. The mass displaced and stretched the peripheral branch of the middle hepatic artery around which abnormal vessels clustered. In the parenchymal phase, macular pooling of contrast material alone was noted, while no densely stained tumor was observed (Fig. 4b). Postarterial porto graphy showed that a branch in the left medial segment was compressed, but stenosis or occlusion was absent up to the second bifurcation. US-guided percutaneous find Internal

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capsulated. The cut surface was largely brownish black and partly yellowish white (Fig. 5). There was bleeding, but neither necrosis nor septum was recognized within the tumor. Histological examinations of the tissue re vealed mesenchymal hamartoma which consisted of the lymphangiomatous tissue with hemorrhage (Fig. 6a), proliferation of the fibrous tissue containing a bile duct element (Fig. 6b), extramedullary hematopoietic foci (Fig. 6c), and clusters of hepatocytes at the periphery of the lesion. No cystic degeneration was noted. The

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Fig. 4. a) The arterial phase of common hepatic arteriography shows displacement and stretching of the peripheral branch of the middle hepatic artery accompanied by a cluster of moniliform abnormal vessels (arrow) with a slightly curved serpentine flow runnin in some part of the peripheral branch, b) The parenchymal phase shows a macular pooling of contrast material (arrow), but no heavily stained tumor.

Discussion Mesenchymal hamartoma of the liver is an uncommon benign lesion usually found in patients under 2 years of age (1, 2). An adult case of this disease in a 22-year-old female was initially described by Yamamura et al in 1976 (3). Since then, to our knowledge, 14 adult cases prior to this communication have been reported (3-16). Table 1 lists the 14 patients and the present patient. They comprised 4 males and ll females, ranging in age from 19 to 69. Eleven patients were symptomatic, presenting with increasing abdominal distention or abdominal pain. The other 4 asymptomatic patients were discovered to have a hepatic mass incidentally Fig. 5. Cut surface of the resected liver shows a well-demarcated during a routine examination or at surgery. As the tumor, largely brownish black and partly yellowish white. method for confirming tumors, US was used the most with 9 cases, suggesting the significance of US on the screening of hepatic lesions. The tumor location was non-tumor portion of the liver was found to be normal mentioned 14 of cases whichtumors are broken down to Diag left liver parenchyma. The averageinsize reported was 18.0cm. lobe 8 cases, right lobe 3 cases and both lobes 3 cases. At present, one year and ten months after surgery, nosis was established by histopathological examinations the patient is doing well. after surgery in all but one case which was diagnosed at autopsy. Preoperative diagnosis markedly differed according to the pathological difference in tumors, that is, which component is predominant, cystic or solid. 1372

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Mesenchymal Hamartomaof the Liver Making an established diagnosis preoperatively ap peared difficult particularly in tumors in which the solid component was predominant. Hamartoma is thought not to be neoplasm but to be produced congenitally by the aberrant development of primitive mesenchyme in the portal tract (17). Micro scopically, this tumor consists of the connective tissue withcontain bile duct elements and (1, 2, 17). Itmixed may also angiomatous or hepatocytes extramedullary hematopoietic foci (1, 2). Cysts are found in nearly all pediatric cases (2). However, the tumor may present itself as a primarily solid or primarily cystic mass, because the amount of stroma may be variable (18). In fact, in adult cases (Table 1), 7 were of the solid predominance type,US while were of the cystic predominance type. The and 8CT appearances reflect the cystic changes seen pathologically, which vary from a solid mass with no cystic components to a multiloculated cystic mass with few solid septa (6, 9, ll, 13, 18-20). There has been only one report on the magnetic resonance imaging appearance in a case with numerous cysts (21). It showed a multiseptated mass with fluid-filled compartments. Its The angiographic appearance varies from a totally avas appearance may also reflect the pathologic changes. cular mass in cases with cystic predominance to a mass with fine tumor vessels (5, 6, ll, 13, 19, 20). Because imaging characteristics are relatively non specific, the diagnostic possibilities are numerous (18). The differential diagnosis of cystic predominance type in an adult includes simple cyst, hematoma, abscess, echino coccal cyst, biliary cystadenoma, and cystic metastasis. Solitary simple hepatic cyst is generally spherical and internal echoes are usually absent unless it is complicated with hemorrhage or infection (22). Hematoma contains a variable amount of internal echoes for one month after the development, and then gradually becomes anechoic (23). Hepatic abscess, whether pyogenic or amebic in etiology, occasionally appears septated or multilocular on CT images (24). Echinococcal cyst is commonly multilocular in appearance because of the presence of daughter cysts within the orginal cyst (25). The diagnosis of these infectious diseases is usually easily made by a combination of clinical and laboratory findings. Biliary cystadenoma is a rare bile ductal neoplasm that occurs primarily in middle-aged women and that may present similar US and CT findings (26). However, angiographi Fig. 6. Photomicrographs show a) the lymphangiomatous tissue cally it is less vascular compared to that of mesenchymal consisting of reticular follicles accompanied by hemorrhage (HEhamartoma stain, (27). Cystic metastasis may appear similar xlO), b) proliferation of the fibrous tissue containing a bile duct to benign cystic disease of the liver (28). Percutaneous The differential diagnosis of solid predominance type element (HE stain, xlO), and c) extramedullary hematopoietic foci cyst aspiration would provide helpful information for includes focal nodular hyperplasia, adenoma, cavernous (HE stain, x40). differentiationangiomyolipoma, of metastasis fromhepatocellular primary hepatic hemangioma, car tumor. cinoma, and other malignant hepatic tumors. Hepatic mesenchymal hamartoma in which solid components are Internal

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22 /F

4 K u ro k a w a (19 8 4 )(6 ) 5 Ish izu k a (19 8 5 )(7 ) 6 K aw a k am i (19 8 6)(8 ) 7 Je n n in g s (198 7 )(9 ) 8 K a to (1 98 8 )(10 ) 9 G u tie rre z (1 98 8 )(l l) 10 G ra m lic h ( 19 8 8 )(12 ) l l A la n e n ( 198 9 )(13 ) 12 Ito ( 19 8 9 )(14 ) 13 U ra b e (19 9 O )(15 ) 14 D ra ch e n b e rg (19 9 1)(16 ) 15 P re se n t

4 3 /F

US: ultrasonography,

S ign s & S y m p to m s

A ge /S e x

1 Y a m a m u ra (197 6 )(3 ) 2 G rase s (197 9 )(4 ) 3 (19 D o8o3)(5 le y )

19 /F 2 1 /F

Reported

R ig h t h y p o ch o n d ri ac p ain L e ft u p p e r q u a d ra n t p a in dA iste b d on mtioinna l

Adult Cases of Sesenchymal

C o n firm e d by A u to p s y

2 6 x 2 0 x 13

L

2 2 x 15 x lO

N one

39 /F 69 /F 62 /M

n o t d e scri b e d

R

66 /M

43 /F

R & L

U S

H e p a to m e g aly

20 /F

U S

D u ri n g o p e ratio n fo r g a stric ca n ce r In c re a sin g a bd o m in a l U S g irth In cre asin g ab d o m in a l C T g irth N one U S

n o t d esc ri b e d 30 x 2 8 x 12

P a th o lo gy

M e se n ch y m al ha m a rto m a C y stic d isea se o f th e liv e r H ep a tic c yst

A u to p sy

C y stic

O p e ra tio n

C y stic

O p e ra tio n

C y stic

C av e rn o u s h e m an gio m a su sp e cted no t d e sc ri b ed

O p e ra tio n

S o lid

O p e ra tio n

C ystic

O p e ra tio n

C ystic

O p e ra tio n

C ystic

O p e ra tio n

S o lid

W e d g e live r b io p sy O p e ra tio n O p e ratio n

C y stic & so lid S o lid & cy stic S o lid

O p e ra tio n

C y stic

O p e ra tio n

S o lid

O p e ra tio n

S o lid & cy stic S o lid

n o t d esc ri b e d

L

14

L

no t d e scri b e d

H e p atic m align a n t tu m o r su sp ec te d H y d a tid d ise a se o f th e liv e r no t d esc ri b e d

18

n o t d esc ri b e d

R & L R L

U S

R & L

US

L

A b d o m in a l d iste n tio n N one

CT

L

U S

L

tomography,

E sta b lish e d by

R

A b d o m in al d iste n tio n N one

CT: computed

P re o p era tive (a t a u to p sy ) d iag n o sis

22

3 2 /F

28 /F

S iz e (c m )

L

6 7 /M

30 /F

L o ca tio n (lo b e )

S cin tig rap h y

A b d o m in al U S d iste n tio n In cre asin g a b d o m in al n o t d e sc ri b e d girth H e p ato m e g aly U S

59 /M

Hamartoma of the Liver

3 0 x 2 0 x 14

H e p a tic h e m a n gio m a 5 H e p a tic m a lig n a n t tu m o r su sp e cte d 16 x 1 6 x 7 .7 C ystic d ise a se o f th e liv er 1 .2 A d e n o m a o r fo c al no d u lar h y p e rp la sia su sp ec te d 2 6 x 2 0 x l l .5 C ystad e n o c arcin o m a 6 x 6 x 4 .5

H e p a tic m a lign a n t tu m o r su sp e cted

O p e ra tio n

R: right, L: left.

predominant appears hypervascular frequently on angio graphy, being difficult to differentiate from these dis eases. A fine needle biopsy specimen has no diagnostic role either for mesenchymal hamartoma (4, 13). In the present case, the tumor was macroscopically solid, and The the correct tumor isdiagnosis mostly slow was growing, not made but before rapid surgery. enlarge ment has been reported (7, 29), possibly due to the accumulation of fluid within the cystic component of the tumor. Accordingly, when mesenchymal hamartoma is suspected, themode lesion considered. In 15 cases resection (Table 1),ofthe ofshould therapybewas principally

References Edmondson HA. Differential diagnosis of tumors and tumor like lesions of liver in infancy and childhood. Am J Dis Child 91: 168, 1956. Stocker JT, Ishak KG. Mesenchymal hamartoma of the liver: Report of 30 cases and review of the literature. Pediatr Pathol 1: 245, 1983. Yamamura T, Kameyama Y, Hoshino T, Hayakawa K. A case of mesenchymal hamartoma of the liver coexisting with congenital dilatation of the bile duct (translation of Japanese title). J Jpn Soc Clin Surg 37: 862, 1976 (in Japanese). Grases PJ, Matos-Villalobos M, Arcia-Romero F, Lecuna-Torres V. Mesenchymal hamartoma of the liver. Gastroenterology 76: 1466, 1979. Dooley JS, Li AKC, Scheuer PJ, Hobbs KEF, Sherlock S. A giant cystic mesenchymal hamartoma of the liver: Diagnosis, management, and study of cyst fluid. Gastroenterology 85: 958, 1983. Kurokawa H, Sasaki K, Kamita N, Yamaguchi Y, Kikuchi Y, Kojimahara M. A case of huge hepatic hamartoma. Jpn J Clin Radiol 29: 901, 1984 (in Japanese with English abstract). Ishizuka M, Miyashita M, Takahama M. A case of giant mes enchymal hamartoma of the liver found in an adult (translation of Japanese title). Proc Jpn Cancer Assoc 44: 490, 1985 (Abstract in Japanese). Kawakami A, Fujii H, Ono Y, et al. A case of hepatic mes

resection of the lesion, and in most cases, the resection was successful. Neither malignant transformation nor tumor recurrence has ever been described. With the recent advance of diagnostic modalities such as US and CT for abdominal diseases, chances of discovering tumors and tumor-like lesions of the liver have been increasing. Although hepatic mesenchymal hamartoma is extremely rare in adults, this disorder should be included in the differential diagnosis of tumors with unusual features in the liver. Acknowledgements: We wish to thank Drs. N. Nakao, K. Miura, enchymal hamartoma in an adult (translation of Japanese title). and K, Kang for helpful comments on US and radiographic findings Nippon Act Radiol 46: 1073, 1986 (Abstract in Japanese). Jennings CM, Merrill CR, Slater DN. Case report: The computed and Dr. K. Nishikawa for referring the patient to us. 1374

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Mesenchymal Hamartomaof the Liver tomogaphic appearances of benign hepatic hamartoma. Clin Comput Assist Tomogr 10: 51, 1986. Radiol 38: 103, 1987. Ros PR, Goodman ZD, Ishak KG, et al. Mesenchymal hamartoma Kato Y, Yokozaki H. Hepatic mass (mesenchymal hamartoma) of the liver: Radiologic-pathologic correlation. Radiology 158: (translation of Japanese title). J Hiroshima Med Assoc 41: 143, 619, 1986. 1988 (Abstract in Japanese). Stanley P, Hall TR, Wooley MM, Diament MJ, Gilsanz V, Gutierrez OH, Burgener FA. Mesenchymal hamartoma of the Miller JH. Mesenchymal hamartomas of the liver in childhood: liver in an adult: Radiologic diagnosis. Gastrointest Radiol 13: Sonographic and CT findings. AJR 147: 1035, 1986. 341, 1988. Roberts EA, Liu P, Stringer D, Superina RA, Mancer K. Mesen Gramlich TL, Killough BW, Garvin AJ. Mesenchymal hamartoma chymal hamartoma in a 10-month-old infant: Appearance by of the liver: Report of a case in a 28-year-old. Hum Pathol 19: magnetic resonance imaging. J Can Assoc Radiol 40: 219, 1989. Spiegel RM, King DL, Green WM. Ultrasonography of primary 991, 1988. Alanen A, Katevuo K, Toikkanen S. A non-cystic mesenchymal cysts of the liver. AJR 131: 235, 1978. hamartoma of the liver-an unusual case of an unusual entity. Wicks JD, Silver TM, Bree RL. Gray scale features of hematomas: Case report and review of the literature. Bildgebung 56: 181, 1989. An ultrasonic spectrum. AJR 131: 977, 1978. Ito Y, Seto Y, Okano H, et al. A case ofmesenchymal hamartomaHalvorsen RA, Korobkin M, Foster WL, Silverman PM, of the liver in adult woman with pancreas divism and marked Thompson WM. The valuable CT appearance of hepatic abscesses. delay in ICG plasma clearance. Jpn J Gastroenterol 86: 1534, AJR 141: 941, 1984. Lewall DB, McCorkell SJ. Hepatic echinococcal cysts: Sono 1989 (in Japanese). Urabe S, Furukawa M, Nakata T, et al. A case of mesenchymal graphic appearance and classification. Radiology 155: 773, 1985. hamartoma of the liver in an adult (translation of Japanese title).Choi BI, Lim JH, Han MC, et al. Biliary cystadenoma and Jpn J Gastroenterol Surg 23: 664, 1990 (Abstract in Japanese). cystadenocarcinoma: CT and sonographic findings. Radiology Drachenberg CB, Papadimitriou JC, Rivero MA, Wood C. Adult 171: 57, 1989. mesenchymal hamartoma of the liver: Report of a case with lightForrest ME, Cho KJ, Shields JJ, Wicks JD, Silver TM. Mc microscopic, FNA cytology, immunohistochemistry, and ultra Cormick TL. Biliary cystadenomas: Sonographic-angiographic pathologic correlations. AJR 135: 723, 1980. structural studies and review of the literature. Mod Pathol 4: Federle MP, Filly RA, Moss AA. Cystic hepatic neoplasms: 392, 1991. Dehner LP, Ewing SL, Sumner HW. Infantile mesenchymal Complementary roles of CT and sonography. AJR 136: 345, hamartoma of the liver. Histologic and ultrastructural obser 1981. Ishida M, Tsuchida Y, Saito S, Sawaguchi S. Mesenchymal vations. Arch Pathol 99: 379, 1975. Giyanani VL, Meyers PC, Wolfson JJ. Mesenchymal hamartoma hamartoma of the liver: Case report and literature review. Ann of the liver: Computed tomography and ultrasonography. J Surg 164: 175, 1966.

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Mesenchymal hamartoma of the liver: report of an adult case and review of the literature.

A case of mesenchymal hamartoma of the liver incidentally found to be a hyperechoic mass by abdominal ultrasonography in a 62-year-old male is describ...
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