Japanese Journal of Clinical Oncology 31:119-121 (2001)
© 2001 Foundation for Promotion of Cancer Research
Hepatocellular Carcinoma with Pancoast’s Syndrome as an Initial Symptom: a Case Report
1Department of Internal Medicine, 2Division of Pulmonary Immunology and Infectious Diseases, Chest Department and 3Department of Pathology, Taipei Veterans General Hospital, National Yang-Ming University School of Medicine, Taipei, Taiwan
 |
ABSTRACT |
|---|
 TOP ABSTRACT INTRODUCTIONCASE REPORTDISCUSSIONREFERENCES |
|---|
Pancoast’s syndrome refers to a condition consisting of Horner’s syndrome and arm pain that is most commonly found in patients with a lung tumor of the superior sulcus invading the upper ribs or spine, lower brachial plexus and sympathetic chain. We report a 76-year-old female who had a thoracic inlet mass that presented as Pancoast’s syndrome, showing profound pain, numbness and weakness of the left upper limb. Further evaluation revealed an increased level of serum
-fetoprotein (24 278 ng/ml), cryptogenic liver cirrhosis and primary hepatocellular carcinoma with protruding T3 vertebra metastasis that resulted in Pancoast’s syndrome. To our knowledge, it is a rare case and only one case has been reported previously. |
INTRODUCTION |
|---|
|
TOPABSTRACTINTRODUCTIONCASE REPORTDISCUSSIONREFERENCES |
|---|
Pancoast’s syndrome is most often seen in patients with pulmonary tumors at the apex of the lung, resulting in pain around the shoulder, radiating down the medial aspect of the arm, ipsilateral Horner’s syndrome (ptosis, miosis, enophthalmos and anhydrosis), weakness and atrophy of intrinsic muscles of the hand on the same side (1). We describe here a female patient who presented with Pancoast’s syndrome associated with protruding T3 vertebra metastasis of primary hepatocellular carcinoma (HCC). To our knowledge, Pancoast’s syndrome caused by metastatic HCC is rare and only one case of a male patient has been reported previously (2). This report will help clinicians to recognize the association between Pancoast’s syndrome and HCC.
|
CASE REPORT |
|---|
|
TOPABSTRACTINTRODUCTIONCASE REPORTDISCUSSIONREFERENCES |
|---|
A 76-year-old housewife was admitted to our hospital with the chief complaint of left upper chest and arm pain for 9 months. She had been well before and denied any history of alcohol consumption, blood transfusion or liver disease.
On examination she was afebrile. There were significant left eye miosis and ptosis, but no left side anhydrosis. There was no palpable neck or axilla lymph node. The chest revealed symmetric expansion with a clear breathing sound. The heartbeat was regular without murmur. Abdominal examination revealed no significant findings. The left hand muscle showed decreased strength, without evidence of atrophy. Other neurological examinations were normal.
Nine months before this admission, chest radiography (CXR) and electrocardiography showed negative findings. Four months before admission, a whole-body bone scan revealed moderately increased radioactivity over the T3 vertebra, suggesting degenerative change. One month before admission, she felt numbness, weakness and pain over the medial side of the left upper limb. CXR showed a soft tissue mass over the left upper lobe with local destruction of the posterior part of the second rib (Fig. 1). Chest computerized tomography (CT) revealed a soft tissue mass about 6 cm in diameter over the left upper lobe with erosion of the first three ribs and also revealed a suspicious intraspinal extension at the T3 vertebra body. CT also revealed a more than 4 cm low attenuation area over the right hepatic lobe with splenomegaly and an engorged splenic vein.
|
Laboratory data showed a hemoglobin concentration of 12.3 g/dl, a white blood cell count of 2.6 x 109/l with a normal differential and a platelet count of 7.8 x 1010/l. Alanine aminotransferase was 14 U/l (normal range: 0–40 U/l) and aspartate aminotransferase was 50 U/l (normal range: 5–45 U/l). Alkaline phosphotase,
-glutamyltransferase, total bilirubin and albumin levels were all within normal limits. The prothrombin time was 14.2/11.5 s (international normalized ratio: 1.26) and the activated partial thromboplastin time was 30.2/30.5 s. The serum -fetoprotein (AFP) level was elevated up to 24 278 ng/ml, but the carcinoembryonic antigen (CEA) level was normal. Anti-HCV, HBsAg and anti-HBc IgM were negative, but anti-HBs Ab was positive. The sonogram showed micronodular cirrhotic change of the liver and an ill-defined right hepatic tumor at the anteriosuperior segment (S8) with portal vein thrombosis. Magnetic resonance imaging (MRI) of the upper abdomen also revealed this hepatic tumor with strong contrast enhancement after gadolinium injection. Several adjacent small daughter nodules were also detected. Liver cirrhosis with portal hypertension was evidenced by MRI, which showed a hypertrophic lateral segment of liver and splenomegaly, with marked collateral circulation over the lesser omentum and left retroperitoneum with splenorenal shunt (Fig. 2). The sono-guided lung biopsy revealed HCC with a positive cytoplasmic AFP immunostain (Fig. 3). Both CT-guided and sono-guided liver biopsies failed to obtain a diagnostic pathological tissue.
|
|
Under the impression of primary HCC with lung metastasis, she received palliative radiotherapy to the metastatic area of the left apical lung with a total dose of 35 Gy in 14 fractions. The follow-up CXR showed a slight enlargement of the left apical tumor, even though the patient subjectively felt less pain and numbness in her left arm. The patient was discharged and followed up regularly.
Four months later, the patient developed sudden onset of paraplegia and numbness below T4. MRI of the spine revealed soft tissue masses over vertebral bodies and posterior neural arches of T1 through T6, with a huge left paraspinal extrapleural mass occupying the left upper chest. Direct tumor extension into the spinal canal, with marked extradural compression of spinal arch at T2 through T7 levels, was also observed (Fig. 4). In addition to increased radioactivity in T3, a whole-body bone scan showed new foci in T5, T6 and L1, suggesting metastasis in progress. MRI of the abdomen showed a progressive enlargement of the previous hepatic lesions.
|
The patient then received palliative radiotherapy with a total dose of 30 Gy over the thoracic spine. However, paraplegia still persisted and acute urinary retention developed during therapy. CXR showed progressive enlargement of the left apical mass with a moderate amount of bilateral pleural effusion. Nine months after diagnosis, the patient died due to respiratory failure. Autopsy was not performed.
|
DISCUSSION |
|---|
|
TOPABSTRACTINTRODUCTIONCASE REPORTDISCUSSIONREFERENCES |
|---|
Pancoast’s syndrome is commonly caused by bronchogenic carcinoma. The differential diagnoses include other primary thoracic neoplasms, metastatic, including carcinoma of the larynx, cervix, urinary bladder and thyroid gland and hematological conditions, infectious diseases, neurogenic thoracic outlet syndromes and pulmonary amyloid nodules (3). Pancoast’s syndrome is an unusual manifestation of thoracic involvement of hepatocellular carcinoma. In this patient with the initial presentation of Pancoast’s syndrome, an attempt was made to obtain a confirmatory pathological diagnosis.
The most useful diagnostic criteria of HCC are the similarity of tumor cells to liver cells, the prominence of nuclei, a trabecular pattern of growth and the presence of a sinusoidal stroma (4). Immunohistochemically, HCC has been found to be immunoreactive to AFP, keratin, EMA, 1-antitrypsin, etc. In our patient, the lung biopsy revealed a typical architecture of HCC, grade 3, with a broad trabecular growth pattern, sinusoidal vascular channels and a positive AFP immunostain of tumor cells, so HCC was diagnosed. We suggest that, in those cases where the architecture of HCC is not as obvious as that illustrated in our case, diagnosis can be aided by performing other immunohistochemical stains, such as alkaline phosphatase, CEA or AE13, to clarify the bile canalicular structure.
It has been documented that serum AFP >500 ng/ml in patients with cirrhosis can be diagnosed with the development of HCC (5). Since our patient had a high serum AFP of 24 278 ng/ml, with presence of liver cirrhosis and contrast enhancement on the CT scan, primary HCC was strongly suggested, although diagnostic liver tissue was not obtained.
Elevation of serum AFP levels may have several etiologies. It is also associated with AFP-producing lung cancer, such as hepatoid adenocarcinoma (6) and endodermal sinus tumor. The term ‘hepatoid carcinoma’ has been defined as a type of malignant tumor that arises in extrahepatic sites, but resembles HCC both histologically and immunohistochemically in its staining for AFP. Since our patient had a bone metastasis before the lung lesion and the later MRI also proved that the lung mass resulted from protruding bone metastasis, primary hepatoid lung cancer with liver metastasis was ruled out.
Pulmonary metastases of primary HCC are mainly spread hematogenously, lymphatically or by direct invasion. The common thoracic roentgenological appearances of metastatic HCC are multiple nodulation, lymphangitic carcinomatosis, miliary lesions and pleural effusion (7). The incidence of bone metastasis of primary HCC ranges from 2 to 13% (8–10). The most common sites of bone metastasis are the vertebra and the ribs (10,11). Bone metastasis is usually caused by hematogenous spreading (12). HCC presenting as a chest wall tumor on the chest radiograph associated with Pancoast’s syndrome has been rarely reported. In this case, osteolytic metastasis of HCC resulted in a large protruding mass of tumor that caused Pancoast’s syndrome.
Our patient’s initial clinical presentation was left upper chest and arm pain for months. Owing to a normal CXR, Pancoast’s syndrome was not suspected initially. Although the whole-body bone scan showed a radiouptake over T3 as early evidence of bone metastasis, it was difficult to interpret owing to the single lesion. In such cases, MRI may be helpful in early diagnosis (13). In conclusion, metastatic HCC should be included in the differential diagnosis of Pancoast’s syndrome, especially in patients with liver cirrhosis and elevated serum AFP levels.
|
FOOTNOTES |
|---|
+ For reprints and all correspondence: Wei-Juin Su, Chest Department, Taipei Veterans General Hospital, No. 201, Sec. 2, Shih-Pai Road, Taipei 112, Taiwan. E-mail: wjsu@vghtpe.gov.tw
|
REFERENCES |
|---|
|
TOPABSTRACTINTRODUCTIONCASE REPORTDISCUSSIONREFERENCES |
|---|
1 Pancoast HK. Superior pulmonary sulcus tumour: tumour characterized by pain, Horner’s syndrome, destruction of bone and atrophy of hand muscles. J Am Med Assoc 1932;99:1391–6.
2 Chocarro A, Labanda F, Martinez E,. Pereda JM. Sindrome de Pancoast como primera manifestacion de hepatocarcinoma. Med Clin 1986;86:822.
3 Arcasoy SM, Jett JR. Superior pulmonary sulcus tumors and Pancoast’s syndrome. N Engl J Med 1997;337:1370–6.
4 Barwick KW. Liver. In: Rosai J, editor. Ackerman’s Surgical Pathology. St. Louis, MO: Mosby Yearbook 1996;903–10.
5 Jones DB, Koorey DJ. Screening studies and markers. Gastroenterol Clin N Am 1987;16:563–73.[Medline]
6 Ishikura H, Kanda M, Ito M, Nosaka K, Mizuno K. Hepatoid adenocarcinoma: a distinctive histological subtype of -fetoprotein-producing lung carcinoma. Virchows Arch Pathol Anat Histopathol 1990;417:73–80.
7 Tsai GL, Liu JD, Siauw CP, Chen PH. Thoracic roentgenologic manifestations in primary carcinoma of the liver. Chest 1984;86:430–4.
8 Carnahan DS. Primary hepatoma with metastasis to a long bone. Radiology 1950;55:844–7.
9 Patton RB, Horn RC. Primary liver carcinoma: autopsy study of 60 cases. Cancer 1964;17:757–68.[Medline]
10 Talerman A, Magyar E. Hepatocellular carcinoma presenting with pathological fracture due to bone metastases. Cancer 1973;32:1477–81.[Medline]
11 Greene JM. Primary carcinoma of the liver: a ten year collective review. Surg Gynecol Obstet 1939;69:231–6.
12 Okuda K, Okuda H. Primary liver cell carcinoma. In: Bircher J, Benhamou JP, Bircher J, Rizetto M, Rodés J, editors. Oxford Textbook of Clinical Hepatology. New York: Oxford University Press 1999;1491–1530.
13 Takasugi JE, Rapoport S, Shaw C. Superior sulcus tumors: the role of imaging. J Thorac Imaging 1989;4:41–8.[ISI][Medline]
Received September 25, 2000; accepted December 8, 2000.
CiteULike 
Connotea 
Del.icio.us What's this?
This article has been cited by other articles:
|
|
 |
|
  T W Marjanski, W Rzyman, and J Skokowski Pancoast's syndrome as a result of metastatic renal cell carcinoma Thorax, April 1, 2003; 58(4): 370 - 370. [Full Text] [PDF]
|
|
| ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||