- Email: [email protected]
Isolated pineal region metastasis of small cell lung cancer
Pineal region metastasis of small cell lung cancer 691
21. Watanabe Y, Fukuda T, Araki M, Hiraki S. A case of angiosarcoma of the heart with cardiac tamponade and repeated hemoptysis. Nihon Kyobu Shikkan Gakkai Zasshi 1993; 31: 1485–1488 (Japanese). 22. Ziegler JW. Primary angiosarcoma of the brain: report of a case. J Am Osteopath Assoc 1975; 74: 957–960.
Isolated pineal region metastasis of small cell lung cancer Jung Yong Ahn MD, Young Sun Chung Seong Oh Kwon MD, Ryoong Huh MD, Sang Sup Chung MD
MD,
Department of Neurosurgery, Pundang CHA Hospital, Pochon CHA University, Sungnam, South Korea
Summary The pineal region is an unusual site for brain metastasis and most metastatic pineal lesions are asymptomatic. Rarely the symptoms of metastatic involvement of the pineal gland precede those of the primary tumor or other metastatic sites. An 83 year-old man presented with gait disturbance and limitation of upward gaze. Brain MRI showed homogeneous enhancement of a solitary mass in the pineal region with obstructive hydrocephalus. A stereotactic biopsy was performed, and small cell carcinoma was diagnosed. A systemic investigation for the primary lesion subsequently revealed small cell carcinoma of the lung. The patient was referred for radiotherapy and chemotherapy. Although rare, metastatic tumor should be considered in the differential diagnosis of pineal region tumors, particularly in elderly patients. ª 2005 Elsevier Ltd. All rights reserved.
INTRODUCTION The pineal region is an unusual site for brain metastasis of systemic malignant tumors. Metastasis to the pineal region accounted for only 0.3% of all brain metastasis in a series from Japan1 but ranges from 1.8% to 4% in other series.2–4 A recent literature review identified a total of 87 reported cases. In approximately half of these cases, the pineal gland was the only site of intracranial metastasis.5 Metastases varied in size. The most common site of primary origin is lung carcinoma, followed by breast carcinoma and other malignancies including melanoma, gastric carcinoma, myeloma and tumours of the kidney and colon.6 The mechanism of pineal metastasis is not established, but metastasis via the bloodstream seems most probable. Recently, we treated a patient with a pineal region metastasis, proven to be from a small cell carcinoma of the lung, which was radiosensitive and chemosensitive. Here, we report this patient with an isolated pineal region mass with radiological features mimicking a primary pineal region tumor.
CASE REPORT This 82 year-old man suffered sudden onset severe headache. He had developed progressively worsening gait and visual disturbance 2 months prior to admission. He had a history of hypertension and chronic obstructive pulmonary disease. On examination, he was noted to be hypertensive and confused but obeying commands. Neurological examination revealed cerebellar dysfunction on the left and limitation of upward gaze. A CT scan showed
Journal of Clinical Neuroscience (2005) 12(6), 691–693 0967-5868/$ - see front matter ª 2005 Elsevier Ltd. All rights reserved. doi:10.1016/j.jocn.2004.09.010
Keywords: pineal region, metastasis, lung carcinoma, small cell carcinoma Received 16 July 2004 Accepted 1 September 2004 Correspondence to: Jung Yong Ahn, Department of Neurosurgery, Pundang CHA Hospital 351, Yatap-dong, Pundang-gu, Sungnam, 463-712, South Korea. Tel.: 82 31 780 5263; Fax: +82 31 780 5269; E-mail: [email protected]
Fig. 1 Axial CT scan showing a 2 · 2 cm hyperdense space-occupying lesion in the pineal region and associated ventricular enlargement.
Fig. 2 MRI scans showing the tumor, which is isointense on the T1-weighted sagittal image (a), uniformly hypointense on the T2-weighted axial image (b), and shows homogeneous enhancement after administration of gadolinium (c).
ª 2005 Elsevier Ltd. All rights reserved.
Journal of Clinical Neuroscience (2005) 12(6)
692 Ahn et al.
hydrocephalus and a 2 · 2 cm hyperdense space-occupying lesion in the pineal region which enhanced after contrast administration (Fig. 1). Subsequently, MRI demonstrated the tumor to be isointense on T1-weighted images, uniformly hypointense on T2-weighted images and to enhance homogeneously after administration of gadolinium (Fig. 2). The tumor margins were well defined and the brainstem was mildly compressed. There were no other intracranial space-occupying lesions. Serum alpha-fetoprotein and beta-human chorionic gonadotropin were within normal limits. A stereotactic biopsy was performed, and a diagnosis of small cell carcinoma was established (Fig. 3). Tumor cells were immunopositive for cytokeratin but not for synaptophysin, neuron specific enolase, or chromogranin A. Systemic investigation for a primary lesion using fluorine-18 fluorodeoxyglucose positron emission tomography (FDG-PET) scan and chest CT scan revealed a small cell carcinoma of the lung. The patient received whole brain radiation to a total dose of 3,600 cGy. During radiotherapy, his limitation of upward gaze gradually improved, but did not resolve completely. On followup CT scan, the mass was no longer visible and the obstructive hydrocephalus had resolved (Fig. 4). He was subsequently referred for chemotherapy (cyclophosphamide, vincristine, and adriamycin), but died of pulmonary complications 5 months after his initial presentation. Autopsy was refused by the family.
Fig. 3 Histopathology of the surgical specimen showing tumor cells with marked pleiomorphism and multiple large nucleoli, characteristic of small cell carcinoma (Hematoxylin-Eosin · 400).
Fig. 4 Follow-up CT scan showing no tumor and resolution of the hydrocephalus.
Journal of Clinical Neuroscience (2005) 12(6)
DISCUSSION Metastases to the pineal region have been considered very uncommon since the first, from a carcinoma of the lung, was reported by Fçrster in 1858.7 According to Lauro et al,8 83 cases of pineal gland metastasis had been reported by 2000 and 4 have been added between 2000 and 2003.5,8–10 In a large proportion of older cases, pineal metastasis was found at autopsy,2,4,5,11 but more recently, CT and/or MRI has detected such lesions.5,6,8,9,12–17 Ortega et al found a 4% incidence of pineal gland metastasis in a cohort of 130 unselected autopsies.4 In a large survey of 7807 intracranial metastatic tumors in Japan, metastases to the pineal region were found in 23 patients, with an estimated frequency of 0.3%,1 and cases constituting a solitary mass, detectable by CT/MRI, were even less frequent. The primary tumour sites were lung (22 cases) and breast (9 cases). Less frequently pineal metastasis results from malignancies of the gastrointestinal tract (esophagus 2, stomach 3, colorectal 1), skin (melanoma 5), hematologic system (plasma cell leukemia 1, lymphoma 1, multiple myeloma 2), kidney (clear cell carcinoma 1) and frontal sinus (1 case). There were no other metastases within the brain in approximately half of the cases.18 In metastasis arising from the lung, small cell carcinoma12–14,17,19 and undifferentiated carcinoma4,6,11 are the most frequent histolopathological types, although squamous cell carcinoma11 and adenocarcinoma4,5 have also been reported. The route by which metastatic malignant cells reach the pineal region is unclear. Ortega et al. suggested hematogenous spread to the pineal body through the posterior choroidal arteries.4 The differential diagnosis of a pineal region mass in a patient over 60 years of age would include the various histological types of primary pineal tumors18, however, metastasis should also be considered, particularly in elderly patients and those with a history of malignancy. In patients with a known history of malignancy, approximately 90% of supratentorial lesions represent metastasis.20 However, occasionally the symptoms of the metastatic lesion precede those of the primary tumor, as demonstrated by our case. Stereotactic biopsy rather than open surgery in advanced age may be adequate investigation of a pineal region mass.21–23 However, when only small fragments of tumor specimen are obtained, it may be difficult to differentiate between metastatic carcinoma and a carcinoma component of primary pineal teratoma with malignant transformation. Microscopically, small cell carcinomas need to be distinguished from pineoblastoma, which occurs more commonly in the first two decades of life. These tumors can be readily distinguished by immunohistochemical markers: metastatic small cell carcinomas display immunoreactivity for cytokeratins, and pineoblastomas express neuronal and neuroendocrine markers. Brain metastases in small cell lung cancer are frequent, both at diagnosis and as a secondary event. Autopsy series have found brain metastasis in 50% to 70% of patients with small cell lung cancer.24 In early clinical studies, up to 20% of patients were reported to developed isolated brain metastasis.25 More recent series have reported higher rates, in the range of 45% to 54% at 2 to 3 years.26 This is likely to be partly due to better diagnostic imaging, detecting brain metastases which would previously have been ‘‘subclinical.’’ Brain metastases are responsible for substantial morbidity. They can be associated with serious functional impairment, thus diminishing quality of life. Radiation therapy is an important component of the management of small cell lung cancer, which is a relatively radiosensitive tumor.27 Other treatment options including surgery and subsequent radiation therapy, whole brain radiation therapy with concurrent chemotherapy, and stereotactic radiosurgery, which is now generally recommended for isolated brain metastasis of
ª 2005 Elsevier Ltd. All rights reserved.
Marjolin’s ulcer invading scalp dura and brain 693
small cell lung cancer. In our case, radiation therapy with concurrent chemotherapy was recommended rather than surgery due to the patient’s advanced age and poor medical condition. Stereotactic radiosurgery may have been an alternative treatment method. The pineal mass responded well to radiation therapy, disappearing on follow-up CT scan. In conclusion, small cell lung cancer metastasis to the pineal region is rare but may occur in elderly patients or those with a known history of lung cancer. It should be considered in the differential diagnosis of pineal region tumors in such patients. Careful examination, including for systemic malignant disease and extracranial radiological studies will be needed. We consider stereotactic biopsy to be a useful diagnostic procedure at initial presentation of a pineal lesion in elderly patients without history of malignancy. Active management of metastatic small cell carcinoma of the pineal region may improve the quality of life as small cell carcinoma is relatively radiosensitive and chemosensitive.
REFERENCES 1. The Committee of Brain Tumor Registry of Japan. Report of Brain Tumor Statistics in Japan (1969-1993). 10th edn. Neurol Med Chir (Tokyo) 2000; 40 (Suppl): 24. 2. Chason JL, Walker FB, Landers JW. Metastatic carcinoma in the central nervous system and dorsal root ganglia. Cancer 1963; 16: 781–787. 3. France LH. Contribution to the study of 150 cases of cerebral metastases. J Neurosurg Sci 1975; 4: 189–210. 4. Ortega P, Malamud N, Shimkin MB. Metastasis of carcinoma to the pineal body. Arch Pathol 1951; 52: 518–528. 5. Kakita A, Kobayashi K, Aoki N, Eguchi I, Morita T, Takahashi H. Lung carcinoma metastasis presenting as a pineal region tumor. Neuropathology 2003; 23: 57–60. 6. Vaquero J, Martinez R, Magallon R, Ramiro J. Intracranial metastases to the pineal region. Report of three cases. J Neurosurg Sci 1991; 35: 55–57. 7. Fçrster. Ein fall von markschwamm mit ungewohnlich vielfacher metastatischer verbreitung. Arch Path Anat 1858; 13: 271–274. 8. Lauro S, Trasatti L, Capalbo C, Mingazzini PL, Vecchione A, Bosman C. Unique pineal gland metastasis of clear cell renal carcinoma: case report and review of the literature. Anticancer Res 2003; 22: 3077–3080. 9. Kanai H, Yamada K, Aihara N, Watanabe K. Pineal region metastasis appearing as hypointensity on T2-weighted magnetic resonance imaging-case report. Neurol Med Chir (Tokyo) 2000; 40: 283–286. 10. Pantanowitz L, Freedman SJ, Dezube BJ, Joseph JT. November 2002: a 72year-old woman with a pineal gland mass. Brain Pathol 2003; 13: 235–236. 11. Halpert B, Erickson EE, Fields WS. Intracranial involvement from carcinoma of the lung. Arch Pathol 1960; 69: 93–103. 12. Brasseur P, Sukkarieh F, Dupont H, Brohee D. Pineal body metastasis. J Belge Radiol 1994; 77: 162–163. 13. Kashiwagi S, Hatano M, Yokoyama T. Metastatic small cell carcinoma to the pineal body: Case report. Neurosurgery 1989; 25: 810–813. 14. Keyaki A, Makita Y, Nabeshima S, et al. Pineal metastatic tumor from lung cancer initially causing neurological abnormalities of pineal body tumor. No Shinkei Geka 1989; 17: 495–499. 15. Ramina R, Coelho Neto M, Mariushi WM, Arruda WO. Pineal metastasis as first clinical manifestation of colorectal adenocarcinoma. Case report. Arq Neuropsiquiatr 1999; 57: 92–95. 16. Schuster JM, Rostomily RC, Hahn C, Winn HR. Two cases of esophageal carcinoma metastatic to the pineal region with a review of the literature. Surg Neurol 1998; 49: 100–103. 17. Weber P, Shepard KV, Vijayakumar S. Metastases to pineal gland. Cancer 1989; 63: 164–165. 18. Hirato J, Nakazato Y. Pathology of pineal region tumors. J Neurooncol 2001; 54: 239–249. 19. Ouyang R, Rozdilsky B. Metastases of carcinoma to the pineal body. Arch Neurol 1966; 15: 399–403. 20. Voorhies R, Sunaresan N, Thaler H. The single supratentorial lesion. An evaluation of preoperative diagnostic tests. J Neurosurg 1980; 53: 364–368. 21. Kreth FW, Schatz CH, Pagenstecher A, Faist M, Volk B, Ostertag CB. Stereotactic management of lesions of the pineal region. Neurosurgery 1996; 39: 280–291. 22. Regis J, Bouillot P, Rouby-Volot F, Figarella-Branger D, Dufour H, Peragut JC. Pineal region tumors and the role of stereotactic biopsy. Review of the
ª 2005 Elsevier Ltd. All rights reserved.
23. 24.
25.
26. 27.
mortality, morbidity, and diagnostic rates in 370 cases. Neurosurgery 1996; 39: 907–914. Pecker J, Scarabin JM, Vallee B, Brucher JM. Treatment in tumours of the pineal region. Value of stereotaxic biopsy. Surg Neurol 1979; 12: 341–348. Hirsch FR, Paulson OB, Hansen HH, Vraa-Jensen J. Intracranial metastases in small cell carcinoma of the lung: Correlation of clinical and autopsy findings. Cancer 1982; 50: 2433–2437. Rosen ST, Makuch RW, Lichter AS, et al. Role of prophylactic cranial irradiation in prevention of central nervous system metastases in small cell lung cancer. Potential benefit restricted to patients with complete response. Am J Med 1983; 74: 615–624. Vins EF, Le Pchoux C, Arriagada R. Prophylactic cranial irradiation in small cell lung cancer. Semin Oncol 2003; 30: 38–46. Krarup M, Poulsen HS, Spang-Thomsen M. Cellular radiosensitivity of smallcell lung cancer cell lines. Int J Radiat Oncol Biol Phys 1997; 38: 191–196.
Marjolin’s ulcer of the scalp invading calvarial bone, dura and brain Sanjeev K Gupta1 MCH, RK Sandhir2 MCH, Awadhesh K Jaiswal1 MCH, Sushil Kumar1 1
Department of Neurosurgery, Hospital, New Delhi, India
2
MCH
Department of Plastic Surgery, St. Stephens
Summary A Marjolin’s ulcer arising in a chronic scalp scar is reported. Malignant transformation occurred after four decades. The tumour had invaded the skull, dura mater and underlying brain tissue. This case illustrates the importance of appropriate and expeditious initial wound management of scar ulcers with high malignant potential to prevent this devastating outcome and also the role of reconstructive surgical techniques in radical surgery for scalp cancers. ª 2005 Elsevier Ltd. All rights reserved. Journal of Clinical Neuroscience (2005) 12(6), 693–696 0967-5868/$ - see front matter ª 2005 Elsevier Ltd. All rights reserved. doi:10.1016/j.jocn.2004.08.030
Keywords: marjolin’s ulcer, skull, scalp, brain, squamous cell carcinoma Received 1 June 2004 Accepted 10 August 2004 Correspondence to: Dr Sanjeev K. Gupta, 768-A, Barafkhana, Jawahar Nagar Road, Jaipur, 302004, India, Tel.: +91 11 22120479; E-mail: [email protected]
INTRODUCTION Malignant transformation in a non-healing scar has been reported in a wide variety of chronic inflammatory conditions, however the pathophysiology remains unclear. These lesions are frequently overlooked and often inadequately treated. All parts of body can be affected, but the extremities, trunk and scalp are common sites. Development of malignancy is usually slow and squamous cell carcinoma is the major histological type of malignancy.1 We report the occurrence of an aggressive Marjolin’s ulcer in a chronic scar of the scalp following an avulsion injury 42 years previously. The malignant transformation involved not only the entire thickness of the scalp but also invaded and destroyed the underlying calvarial bone with infiltration of the dura and underlying brain. We discuss the pathophysiology of epidermal scar cancers and management strategies for both their prevention and radical excision.
Journal of Clinical Neuroscience (2005) 12(6)
21. Watanabe Y, Fukuda T, Araki M, Hiraki S. A case of angiosarcoma of the heart with cardiac tamponade and repeated hemoptysis. Nihon Kyobu Shikkan Gakkai Zasshi 1993; 31: 1485–1488 (Japanese). 22. Ziegler JW. Primary angiosarcoma of the brain: report of a case. J Am Osteopath Assoc 1975; 74: 957–960.
Isolated pineal region metastasis of small cell lung cancer Jung Yong Ahn MD, Young Sun Chung Seong Oh Kwon MD, Ryoong Huh MD, Sang Sup Chung MD
MD,
Department of Neurosurgery, Pundang CHA Hospital, Pochon CHA University, Sungnam, South Korea
Summary The pineal region is an unusual site for brain metastasis and most metastatic pineal lesions are asymptomatic. Rarely the symptoms of metastatic involvement of the pineal gland precede those of the primary tumor or other metastatic sites. An 83 year-old man presented with gait disturbance and limitation of upward gaze. Brain MRI showed homogeneous enhancement of a solitary mass in the pineal region with obstructive hydrocephalus. A stereotactic biopsy was performed, and small cell carcinoma was diagnosed. A systemic investigation for the primary lesion subsequently revealed small cell carcinoma of the lung. The patient was referred for radiotherapy and chemotherapy. Although rare, metastatic tumor should be considered in the differential diagnosis of pineal region tumors, particularly in elderly patients. ª 2005 Elsevier Ltd. All rights reserved.
INTRODUCTION The pineal region is an unusual site for brain metastasis of systemic malignant tumors. Metastasis to the pineal region accounted for only 0.3% of all brain metastasis in a series from Japan1 but ranges from 1.8% to 4% in other series.2–4 A recent literature review identified a total of 87 reported cases. In approximately half of these cases, the pineal gland was the only site of intracranial metastasis.5 Metastases varied in size. The most common site of primary origin is lung carcinoma, followed by breast carcinoma and other malignancies including melanoma, gastric carcinoma, myeloma and tumours of the kidney and colon.6 The mechanism of pineal metastasis is not established, but metastasis via the bloodstream seems most probable. Recently, we treated a patient with a pineal region metastasis, proven to be from a small cell carcinoma of the lung, which was radiosensitive and chemosensitive. Here, we report this patient with an isolated pineal region mass with radiological features mimicking a primary pineal region tumor.
CASE REPORT This 82 year-old man suffered sudden onset severe headache. He had developed progressively worsening gait and visual disturbance 2 months prior to admission. He had a history of hypertension and chronic obstructive pulmonary disease. On examination, he was noted to be hypertensive and confused but obeying commands. Neurological examination revealed cerebellar dysfunction on the left and limitation of upward gaze. A CT scan showed
Journal of Clinical Neuroscience (2005) 12(6), 691–693 0967-5868/$ - see front matter ª 2005 Elsevier Ltd. All rights reserved. doi:10.1016/j.jocn.2004.09.010
Keywords: pineal region, metastasis, lung carcinoma, small cell carcinoma Received 16 July 2004 Accepted 1 September 2004 Correspondence to: Jung Yong Ahn, Department of Neurosurgery, Pundang CHA Hospital 351, Yatap-dong, Pundang-gu, Sungnam, 463-712, South Korea. Tel.: 82 31 780 5263; Fax: +82 31 780 5269; E-mail: [email protected]
Fig. 1 Axial CT scan showing a 2 · 2 cm hyperdense space-occupying lesion in the pineal region and associated ventricular enlargement.
Fig. 2 MRI scans showing the tumor, which is isointense on the T1-weighted sagittal image (a), uniformly hypointense on the T2-weighted axial image (b), and shows homogeneous enhancement after administration of gadolinium (c).
ª 2005 Elsevier Ltd. All rights reserved.
Journal of Clinical Neuroscience (2005) 12(6)
692 Ahn et al.
hydrocephalus and a 2 · 2 cm hyperdense space-occupying lesion in the pineal region which enhanced after contrast administration (Fig. 1). Subsequently, MRI demonstrated the tumor to be isointense on T1-weighted images, uniformly hypointense on T2-weighted images and to enhance homogeneously after administration of gadolinium (Fig. 2). The tumor margins were well defined and the brainstem was mildly compressed. There were no other intracranial space-occupying lesions. Serum alpha-fetoprotein and beta-human chorionic gonadotropin were within normal limits. A stereotactic biopsy was performed, and a diagnosis of small cell carcinoma was established (Fig. 3). Tumor cells were immunopositive for cytokeratin but not for synaptophysin, neuron specific enolase, or chromogranin A. Systemic investigation for a primary lesion using fluorine-18 fluorodeoxyglucose positron emission tomography (FDG-PET) scan and chest CT scan revealed a small cell carcinoma of the lung. The patient received whole brain radiation to a total dose of 3,600 cGy. During radiotherapy, his limitation of upward gaze gradually improved, but did not resolve completely. On followup CT scan, the mass was no longer visible and the obstructive hydrocephalus had resolved (Fig. 4). He was subsequently referred for chemotherapy (cyclophosphamide, vincristine, and adriamycin), but died of pulmonary complications 5 months after his initial presentation. Autopsy was refused by the family.
Fig. 3 Histopathology of the surgical specimen showing tumor cells with marked pleiomorphism and multiple large nucleoli, characteristic of small cell carcinoma (Hematoxylin-Eosin · 400).
Fig. 4 Follow-up CT scan showing no tumor and resolution of the hydrocephalus.
Journal of Clinical Neuroscience (2005) 12(6)
DISCUSSION Metastases to the pineal region have been considered very uncommon since the first, from a carcinoma of the lung, was reported by Fçrster in 1858.7 According to Lauro et al,8 83 cases of pineal gland metastasis had been reported by 2000 and 4 have been added between 2000 and 2003.5,8–10 In a large proportion of older cases, pineal metastasis was found at autopsy,2,4,5,11 but more recently, CT and/or MRI has detected such lesions.5,6,8,9,12–17 Ortega et al found a 4% incidence of pineal gland metastasis in a cohort of 130 unselected autopsies.4 In a large survey of 7807 intracranial metastatic tumors in Japan, metastases to the pineal region were found in 23 patients, with an estimated frequency of 0.3%,1 and cases constituting a solitary mass, detectable by CT/MRI, were even less frequent. The primary tumour sites were lung (22 cases) and breast (9 cases). Less frequently pineal metastasis results from malignancies of the gastrointestinal tract (esophagus 2, stomach 3, colorectal 1), skin (melanoma 5), hematologic system (plasma cell leukemia 1, lymphoma 1, multiple myeloma 2), kidney (clear cell carcinoma 1) and frontal sinus (1 case). There were no other metastases within the brain in approximately half of the cases.18 In metastasis arising from the lung, small cell carcinoma12–14,17,19 and undifferentiated carcinoma4,6,11 are the most frequent histolopathological types, although squamous cell carcinoma11 and adenocarcinoma4,5 have also been reported. The route by which metastatic malignant cells reach the pineal region is unclear. Ortega et al. suggested hematogenous spread to the pineal body through the posterior choroidal arteries.4 The differential diagnosis of a pineal region mass in a patient over 60 years of age would include the various histological types of primary pineal tumors18, however, metastasis should also be considered, particularly in elderly patients and those with a history of malignancy. In patients with a known history of malignancy, approximately 90% of supratentorial lesions represent metastasis.20 However, occasionally the symptoms of the metastatic lesion precede those of the primary tumor, as demonstrated by our case. Stereotactic biopsy rather than open surgery in advanced age may be adequate investigation of a pineal region mass.21–23 However, when only small fragments of tumor specimen are obtained, it may be difficult to differentiate between metastatic carcinoma and a carcinoma component of primary pineal teratoma with malignant transformation. Microscopically, small cell carcinomas need to be distinguished from pineoblastoma, which occurs more commonly in the first two decades of life. These tumors can be readily distinguished by immunohistochemical markers: metastatic small cell carcinomas display immunoreactivity for cytokeratins, and pineoblastomas express neuronal and neuroendocrine markers. Brain metastases in small cell lung cancer are frequent, both at diagnosis and as a secondary event. Autopsy series have found brain metastasis in 50% to 70% of patients with small cell lung cancer.24 In early clinical studies, up to 20% of patients were reported to developed isolated brain metastasis.25 More recent series have reported higher rates, in the range of 45% to 54% at 2 to 3 years.26 This is likely to be partly due to better diagnostic imaging, detecting brain metastases which would previously have been ‘‘subclinical.’’ Brain metastases are responsible for substantial morbidity. They can be associated with serious functional impairment, thus diminishing quality of life. Radiation therapy is an important component of the management of small cell lung cancer, which is a relatively radiosensitive tumor.27 Other treatment options including surgery and subsequent radiation therapy, whole brain radiation therapy with concurrent chemotherapy, and stereotactic radiosurgery, which is now generally recommended for isolated brain metastasis of
ª 2005 Elsevier Ltd. All rights reserved.
Marjolin’s ulcer invading scalp dura and brain 693
small cell lung cancer. In our case, radiation therapy with concurrent chemotherapy was recommended rather than surgery due to the patient’s advanced age and poor medical condition. Stereotactic radiosurgery may have been an alternative treatment method. The pineal mass responded well to radiation therapy, disappearing on follow-up CT scan. In conclusion, small cell lung cancer metastasis to the pineal region is rare but may occur in elderly patients or those with a known history of lung cancer. It should be considered in the differential diagnosis of pineal region tumors in such patients. Careful examination, including for systemic malignant disease and extracranial radiological studies will be needed. We consider stereotactic biopsy to be a useful diagnostic procedure at initial presentation of a pineal lesion in elderly patients without history of malignancy. Active management of metastatic small cell carcinoma of the pineal region may improve the quality of life as small cell carcinoma is relatively radiosensitive and chemosensitive.
REFERENCES 1. The Committee of Brain Tumor Registry of Japan. Report of Brain Tumor Statistics in Japan (1969-1993). 10th edn. Neurol Med Chir (Tokyo) 2000; 40 (Suppl): 24. 2. Chason JL, Walker FB, Landers JW. Metastatic carcinoma in the central nervous system and dorsal root ganglia. Cancer 1963; 16: 781–787. 3. France LH. Contribution to the study of 150 cases of cerebral metastases. J Neurosurg Sci 1975; 4: 189–210. 4. Ortega P, Malamud N, Shimkin MB. Metastasis of carcinoma to the pineal body. Arch Pathol 1951; 52: 518–528. 5. Kakita A, Kobayashi K, Aoki N, Eguchi I, Morita T, Takahashi H. Lung carcinoma metastasis presenting as a pineal region tumor. Neuropathology 2003; 23: 57–60. 6. Vaquero J, Martinez R, Magallon R, Ramiro J. Intracranial metastases to the pineal region. Report of three cases. J Neurosurg Sci 1991; 35: 55–57. 7. Fçrster. Ein fall von markschwamm mit ungewohnlich vielfacher metastatischer verbreitung. Arch Path Anat 1858; 13: 271–274. 8. Lauro S, Trasatti L, Capalbo C, Mingazzini PL, Vecchione A, Bosman C. Unique pineal gland metastasis of clear cell renal carcinoma: case report and review of the literature. Anticancer Res 2003; 22: 3077–3080. 9. Kanai H, Yamada K, Aihara N, Watanabe K. Pineal region metastasis appearing as hypointensity on T2-weighted magnetic resonance imaging-case report. Neurol Med Chir (Tokyo) 2000; 40: 283–286. 10. Pantanowitz L, Freedman SJ, Dezube BJ, Joseph JT. November 2002: a 72year-old woman with a pineal gland mass. Brain Pathol 2003; 13: 235–236. 11. Halpert B, Erickson EE, Fields WS. Intracranial involvement from carcinoma of the lung. Arch Pathol 1960; 69: 93–103. 12. Brasseur P, Sukkarieh F, Dupont H, Brohee D. Pineal body metastasis. J Belge Radiol 1994; 77: 162–163. 13. Kashiwagi S, Hatano M, Yokoyama T. Metastatic small cell carcinoma to the pineal body: Case report. Neurosurgery 1989; 25: 810–813. 14. Keyaki A, Makita Y, Nabeshima S, et al. Pineal metastatic tumor from lung cancer initially causing neurological abnormalities of pineal body tumor. No Shinkei Geka 1989; 17: 495–499. 15. Ramina R, Coelho Neto M, Mariushi WM, Arruda WO. Pineal metastasis as first clinical manifestation of colorectal adenocarcinoma. Case report. Arq Neuropsiquiatr 1999; 57: 92–95. 16. Schuster JM, Rostomily RC, Hahn C, Winn HR. Two cases of esophageal carcinoma metastatic to the pineal region with a review of the literature. Surg Neurol 1998; 49: 100–103. 17. Weber P, Shepard KV, Vijayakumar S. Metastases to pineal gland. Cancer 1989; 63: 164–165. 18. Hirato J, Nakazato Y. Pathology of pineal region tumors. J Neurooncol 2001; 54: 239–249. 19. Ouyang R, Rozdilsky B. Metastases of carcinoma to the pineal body. Arch Neurol 1966; 15: 399–403. 20. Voorhies R, Sunaresan N, Thaler H. The single supratentorial lesion. An evaluation of preoperative diagnostic tests. J Neurosurg 1980; 53: 364–368. 21. Kreth FW, Schatz CH, Pagenstecher A, Faist M, Volk B, Ostertag CB. Stereotactic management of lesions of the pineal region. Neurosurgery 1996; 39: 280–291. 22. Regis J, Bouillot P, Rouby-Volot F, Figarella-Branger D, Dufour H, Peragut JC. Pineal region tumors and the role of stereotactic biopsy. Review of the
ª 2005 Elsevier Ltd. All rights reserved.
23. 24.
25.
26. 27.
mortality, morbidity, and diagnostic rates in 370 cases. Neurosurgery 1996; 39: 907–914. Pecker J, Scarabin JM, Vallee B, Brucher JM. Treatment in tumours of the pineal region. Value of stereotaxic biopsy. Surg Neurol 1979; 12: 341–348. Hirsch FR, Paulson OB, Hansen HH, Vraa-Jensen J. Intracranial metastases in small cell carcinoma of the lung: Correlation of clinical and autopsy findings. Cancer 1982; 50: 2433–2437. Rosen ST, Makuch RW, Lichter AS, et al. Role of prophylactic cranial irradiation in prevention of central nervous system metastases in small cell lung cancer. Potential benefit restricted to patients with complete response. Am J Med 1983; 74: 615–624. Vins EF, Le Pchoux C, Arriagada R. Prophylactic cranial irradiation in small cell lung cancer. Semin Oncol 2003; 30: 38–46. Krarup M, Poulsen HS, Spang-Thomsen M. Cellular radiosensitivity of smallcell lung cancer cell lines. Int J Radiat Oncol Biol Phys 1997; 38: 191–196.
Marjolin’s ulcer of the scalp invading calvarial bone, dura and brain Sanjeev K Gupta1 MCH, RK Sandhir2 MCH, Awadhesh K Jaiswal1 MCH, Sushil Kumar1 1
Department of Neurosurgery, Hospital, New Delhi, India
2
MCH
Department of Plastic Surgery, St. Stephens
Summary A Marjolin’s ulcer arising in a chronic scalp scar is reported. Malignant transformation occurred after four decades. The tumour had invaded the skull, dura mater and underlying brain tissue. This case illustrates the importance of appropriate and expeditious initial wound management of scar ulcers with high malignant potential to prevent this devastating outcome and also the role of reconstructive surgical techniques in radical surgery for scalp cancers. ª 2005 Elsevier Ltd. All rights reserved. Journal of Clinical Neuroscience (2005) 12(6), 693–696 0967-5868/$ - see front matter ª 2005 Elsevier Ltd. All rights reserved. doi:10.1016/j.jocn.2004.08.030
Keywords: marjolin’s ulcer, skull, scalp, brain, squamous cell carcinoma Received 1 June 2004 Accepted 10 August 2004 Correspondence to: Dr Sanjeev K. Gupta, 768-A, Barafkhana, Jawahar Nagar Road, Jaipur, 302004, India, Tel.: +91 11 22120479; E-mail: [email protected]
INTRODUCTION Malignant transformation in a non-healing scar has been reported in a wide variety of chronic inflammatory conditions, however the pathophysiology remains unclear. These lesions are frequently overlooked and often inadequately treated. All parts of body can be affected, but the extremities, trunk and scalp are common sites. Development of malignancy is usually slow and squamous cell carcinoma is the major histological type of malignancy.1 We report the occurrence of an aggressive Marjolin’s ulcer in a chronic scar of the scalp following an avulsion injury 42 years previously. The malignant transformation involved not only the entire thickness of the scalp but also invaded and destroyed the underlying calvarial bone with infiltration of the dura and underlying brain. We discuss the pathophysiology of epidermal scar cancers and management strategies for both their prevention and radical excision.
Journal of Clinical Neuroscience (2005) 12(6)