中華癌醫會誌 (J. Chinese Oncol. Soc.) 25(6), 454-460, 2009 Case Report journal homepage:www.cos.org.tw/web/index.asp Suprasellar Metastasis of Pulmonary Adenocarcinoma Jen-Ho Tseng 1, Sheng-Huang Hsiao 1, Jung-Mao Chou 2, Ann-Jeng Liu 1 * 1 Department of Neurosurgery, Taipei City Hospital Ren-Ai Branch, Taiwan 2 Department of Pathology, Taipei City Hospital Ren-Ai Branch, Taiwan 病例報告 Abstract. An originally fit and well man suffered from acute headache and loss of consciousness. A hyperdense suprasellar tumor and intraventricular hemorrhage (IVH) were detected by head CT. Cerebrovascular imaging studies excluded the presence of supraclinoid giant aneurysm. An emergency excision of the suprasellar tumor was done for intratumoral hemorrhage, signs of pituitary apoplexy, and IVH. Surgical pathology revealed a metastatic adenocarcinoma from the lung, and pulmonary adenocarcinoma was confirmed following whole-body cancer work-up. This is the first case report of pure suprasellar metastasis as the initial manifestation of an unknown primary tumor. Keywords : suprasellar, metastasis, pulmonary adenocarcinoma 肺腺癌轉移至蝶鞍上部 曾仁河 1 蕭勝煌 1 周榮茂 2 劉安正 1 * 1 台北市立聯合醫院仁愛院區神經外科 2 台北市立聯合醫院仁愛院區病理科 中文摘要一位健康中年男子在一陣突發劇烈頭痛後失去意識, 送來急診後立刻接受腦部電腦斷層掃描, 結果顯示蝶鞍上部有一橢圓形界線清楚的出血性腫塊, 同時有腦室內出血, 電腦斷層腦血管攝影排除腦內頸動脈巨大動脈瘤, 因此, 在腦下垂體大腺瘤併發出血與腦室內出血的情況下, 患者接受緊急開顱手術清除腫瘤, 並引流腦室內血腫, 病理報告顯示此腫瘤為轉移性肺腺癌, 肺部原發病灶隨後也證實為腺癌, 患者意識逐漸復原之後, 接受了腦部放射治療與全身性化學治療 本病例特殊性在於蝶鞍上部的肺腺癌轉移病灶, 以近似腦內頸動脈巨大動脈瘤出血, 與腦下垂體大腺瘤併發出血 ( 腦下垂體中風 ) 來表現 關鍵字 : 蝶鞍上部 轉移 肺腺癌 INTRODUCTION Sellar or suprasellar metastasis as an initial presentation of an unknown primary cancer is rare. We demonstrate a case of lung cancer that initially presented signs of a hemorrhagic supraclinoid giant aneurysm and pituitary apoplexy.
J. H. Tseng et al./jcos 25(2009) 454-460 455 CASE REPORT The 57-year-old man had a 3-month history of headache and duration of general malaise and lethargy for one week. After an acute episode of severe headache, he became comatose with Glasgow Coma Scale (GCS) E1M5Vt following intubation. An emergency computerized tomography (CT) scan of his head disclosed a well-demarcated lesion with a high density in the suprasellar area, subarachnoid hemorrhage, intraventricular hemorrhage (IVH), and acute hydrocephalus (Figure 1). Following insertion of external ventricular drainage, CT angiography (CTA) (Figure 2) and digital subtraction angiography (DSA) (Figure 3) were performed, which excluded a vascular lesion, i.e., a giant thrombolized supraclinoid aneurysm. Serum biochemistry studies showed pituitary insufficiency and elevated carcinoembryonic antigen (CEA) levels. Therefore, under a clinical diagnosis of suprasellar tumor associated with subarachnoid hemorrhage and IVH, the tumor was excised via a standard right pterional craniotomy. The tumor was grossly totally removed under surgical microscope, and there was no tumor invasion into the pituitary fossa. However, the pituitary stalk could not be well identified for intratumoral hemorrhage and adhesion. Histopathology demonstrated features of metastatic adenocarcinoma (Figure 5). The positive result of immunohistochemistry stain with TTF-1 suggested the origin of lung. A further whole-body cancer work-up revealed a left pulmonary tumor and multiple metastatic foci in the skeleton (Figure 4). A bronchoscopic biopsy of the left pulmonary tumor showed an adenocarcinoma similar to that in the suprasellar region (Figure 5). Following the *Corresponding author: Ann-Jeng Liu M.D. * 通訊作者 : 劉安正醫師 Tel: +886-2-27093600 ext.3611 Fax: +886-2-27019975 E-mail: jhtseng3@ms46.hinet.net brain surgery, the patient regained full consciousness with GCS 15. He then underwent a full course of whole-brain irradiation (200 cgy for 20 fractions) and chemotherapy. The patient s treatment course was complicated with recurrent pneumonia, panhypopituitarism, and diabetes insipidus (DI). DISCUSSION The incidence of sellar metastasis ranges from 3% to 5% of patients with carcinoma, and is more common than that of pituitary adenoma. In autopsy studies, micrometastases can be found in as many as 27% of patients who have died of cancer [1]. However, sellar metastasis as the initial presentation of an unknown primary tumor is unusual. There is no predilection of primary cancers metastasizing to the sellar region. Sporadic cases of sellar metastasis have been reported, with the common primary sites being lung, breast, thyroid, kidney (renal cell carcinoma), prostate and a plasmacytoma [1-8]. Mechanisms of the sellar metastasis include hematogenous spread and direct invasion from the skull base. The posterior pituitary is the most common site of metastasis, presumably because of its direct arterial supply compared with the portal circulation of the anterior gland [1]. However, mechanisms of pure suprasellar metastasis are much more complex and include hematogenous spread to the pituitary stalk, and then an extension of the invasion of cancer cells to nearby structures, such as the hypothalamus, median eminence, optic chiasm and lamina terminalis. Deficiency of the blood-brain barrier may be responsible for these invasions. Intracranial micrometastases with cancer cells spreading via cerebrospinal fluid may also account for the suprasellar invasion. Manifestations of sellar metastasis are similar to those of pituitary adenoma, ranging from nonspecific symptoms, such as headache and lethargy, to visual dysfunction and extraocular muscular (EOM) impairment. The abnormality of endocrinology and the presence of diabetes insipidus will alert physicians to
456 J. H. Tseng et al./jcos 25(2009) 454-460 Figure 1. Brain CT without contrast enhancement discloses that, in addition to a well-demarcated hyperdense lesion (2.4 cm; arrowhead in B, C and D) occupying the suprasellar area, there are intraventricular hemorrhage and dilatation of the lateral ventricles. The sellar area (arrow in A) is not involved the occurrence of pituitary dysfunction and organic sellar lesions [1,2,4-6]. Head CT scans can be used to detect large sellar tumors, and is the primary image study for pituitary apoplexy. Magnetic resonance imaging (MRI) can demonstrate more detailed anatomy and characteristics of the sellar tumors. Treatment strategy for the metastatic sellar tumors is the same as for the pituitary adenoma, which can be excised by transsphenoidal approach, and the suprasellar component is excised via pterional approach. Patients presenting signs of pituitary apoplexy need emergency operation. To our knowledge, this is the first case report of pure suprasellar metastasis of pulmonary adenocarci-
J. H. Tseng et al./jcos 25(2009) 454-460 457 Figure 2. CT angiography shows the involvement of suprasellar region by the lesion (coronal section; arrowhead). Asterisk indicates intraventricular hemorrhage Figure 3. A digital subtraction angiography (DSA) does not show aneurysms in the suprasellar area
458 J. H. Tseng et al./jcos 25(2009) 454-460 Figure 4. (A) whole-body CT reveals a small left pulmonary tumor with speculated margins (1.9 cm) (arrow). A bronchoscopic biopsy discloses adenocarcinoma. (B) Tc-99m whole-body bone scan demonstrates multiple metastases involving the vertebral column, rib cage, pelvis, and femurs
J. H. Tseng et al./jcos 25(2009) 454-460 459 Figure 5. (A) excision biopsy of the brain, moderately differentiated adenocarcinoma contains some papillae with fibrovascular cores lined by atypical large cells in the hemorrhagic background (H&E stain, original magnification x 200). Inset, brownish color of nuclei indicates TTF-1 positive and origin of lung (original magnification x 200). (B) core needle biopsy of the lung tumor, moderately differentiated adenocarcinoma contains some glands and papillae. Histological patterns are similar to those from brain biopsy (H&E stain, original magnification x 200) noma in the English literature. It is difficult to make metastatic tumor within optic chiasm: case report. precise clinical diagnosis in the absence of typical Neurol Med Chir (Tokyo) 50: 158-61, 2010. neurological deficits of suprasellar tumor. It is essen- 3. Bobinski M, Greco CM, Schrot RJ. Giant intra- tial to rule out a ruptured supraclinoid giant aneurysm cranial medullary thyroid carcinoma metastasis in this case. The combination of negative cerebrovas- presenting as apoplexy. Skull Base 19(5): 359-62, cular imaging and features of pituitary apoplexy may 2009. lead to the diagnosis of a suprasellar tumor with atyp- 4. Luu ST, Billing K, Crompton JL, et al. Clinico- ical clinical presentation. Morphological studies com- pathological correlation in pituitary gland metas- bined with immunohistochemistry may clarify the na- tasis presenting as anterior visual pathway com- ture of the suprasellar tumor. In conclusion, this was pression. J Clin Neurosci 17(6): 790-3, 2010. an unusual presentation of suprasellar tumor metasta- 5. Peppa M, Papaxoinis G, Xiros N, et al. Panhypo- sized from lung cancer resembling a ruptured supra- pituitarism due to metastases to the hypothalamus clinoid giant aneurysm. The outcome of the sellar or and the pituitary resulting from primary breast suprasellar metastatic cancer depends on the staging cancer: a case report and review of the literature. of the primary cancer, neurological status, and general Clin Breast Cancer 9(4): E4-7, 2009. conditions of the patients during the period of treatment. 6. Riemenschneider MJ, Beseoglu K, Hänggi D, et al. Prostate adenocarcinoma metastasis in the pituitary gland. Arch Neurol 66(8): 1036-7, 2009. REFERENCES 1. Weil RJ, Tenn N. Pituitary metastasis. Arch Neurol 59: 1962-3, 2002. 2. Arai A, Morishita A, Hanada Y, et al. Solitary 7. Weilbaecher C, Patwardhan RV, Fowler M, et al. Metastatic lesions involving the sella: report of three cases and review of the literature. Neurology India 52(3): 365-8, 2004.
460 J. H. Tseng et al./jcos 25(2009) 454-460 8. Xia JL, Wang YS. Papillary thyroid carcinoma metastatic to the pituitary gland: a case report and literature review. J Chin Clin Med 5(2): 116-9, 2010.