Primary Cranial Vault Lymphoma Presenting As a Traumatic Subdural Hematoma Primäres Lymphom der Schädelkalotte präsentiert als traumatisches subdurales Hämatom Abstract Zusammenfassung Objective and Importance: The authors present a rare case of a primary cranial vault lymphoma, mimicking a subacute subdural hematoma after head trauma. Clinical Presentation: A 78year-old woman was admitted to the hospital with progressive left hemiparesis, headache and a gradually increasing soft lump over the right parietal region 1 week after head trauma due to falling from a high place. A computed tomography (CT) scan demonstrated a hyperdense parieto-occipital subdural and subgaleal lesion together with marked midline shift and white matter edema. Intervention: Considering her trauma story and clinical progression, the patient underwent an emergency operation with the diagnosis of subdural hematoma. However, the lesion was found to be of a tumoros nature and the histopathological diagnosis was high-grade malignant non-Hodgkin’s lymphoma. Conclusion: The authors emphasize the rarity of primary cranial vault lymphoma and its importance in the differential diagnosis of cranial vault mass lesions extending either intra- or extracranially. This case may be considered as another example where magnetic resonance imaging (MRI) is the ultimate definitive test in the emergency ward whenever CT demonstrates any findings which are unclear. Die Autoren stellen den seltenen Fall eines primären Schädelkalotten-Lymphoms vor, der in der klinischen Präsentation das Bild eines subakuten Subduralhämatoms nach Schädel-Hirn-Trauma nachahmte. Klinische Präsentation: Eine 78-jährige Frau wurde mit einer progressiven linkseitigen Hemiparese im Krankenhaus aufgenommen. Seit einer Woche, nach einem Sturz aus größerer Höhe, bestanden Kopfschmerzen und eine langsam zunehmende, weiche Schwellung rechts parietal. Der CT zeigte eine hyperdense, subdural und subgaleal gelegene Läsion parietookzipital, verbunden mit deutlicher Mittellinienverlagerung und Marklagerödem. Therapie: Angesicht der Traumavorgeschichte und fortschreitender Symptomatik wurde die Patientin unter der Verdachtsdiagnose eines subduralen Hämatoms operiert. Die Läsion stellte sich jedoch als ein Tumor heraus mit der histologischen Diagnose eines hochmalignen Non-Hodgkin-Lymphoms. Schlussfolgerung: Die Autoren diskutieren die Seltenheit eines primären Kalotten-Lymphoms im Hinblick auf die Bedeutung auf die Differenzialdiagnose von raumfordernden Prozessen der Schädelkalotte, die sich sowohl nach intra- und extrakraniel ausdehnen. Dieser Fall kann auch als weiteres Beispiel dafür betrachtet werden, dass MRT-Untersuchungen die letztlich entscheidende Diagnosemethode auch bei Notfällen darstellt, falls der CT einen unklaren Befund aufzeigt. Key words Elderly · head trauma · primary malignant lymphoma · surgical emergency Schlüsselwörter Schädelhirntrauma · malignes Lymphom · Notfall Affiliation 1 Department of Neurosurgery, Kirikkale University School of Medicine, Kirikkale, Turkey 2 Department of Neurosurgery, Kocaeli University School of Medicine, Kocaeli, Turkey 3 Department of Pathology, Kocaeli University School of Medicine, Kocaeli, Turkey Correspondence Çetin Evliyaoğlu MD · Yılmaz Çolpan Sok. 7/3 · Oran Sitesi · Oran · 06450 Ankara · Turkey · Tel.: +90/318/2 24 00 87 · Fax: +90/318/2 25 2819 · E-mail: cevliyaoglu@hotmail.com Bibliography Zentralbl Neurochir 2006; 67: 26–29 · © J. A. Barth Verlag in Georg Thieme Verlag KG DOI 10.1055/s-2005-872511 ISSN 0044-4251 Downloaded by: WEST VIRGINIA UNIVERSITY. Copyrighted material. Case Report 26 C. Evliyaoğlu1 K. Ilbay2 C. Ercin3 S. Ceylan2 Introduction Primary lymphomas of the central nervous system (CNS) are uncommon, representing merely 0.3 to 1.5 % of all intracranial tumors and 0.9 to 2 % of all malignant lymphomas, although they have been reported with increasing frequency in patients with AIDS [11]. Primary CNS lymphomas may occur at any age or at any site within the brain, but primary cranial vault involvement is extremely rare [1, 5, 8, 9]. We report the case of a primary malignant lymphoma of the skull that had erroneously been diagnosed as a traumatic subdural hematoma due to its clinical presentation and CT imaging features. a b 27 c Fig. 1 a–c a Axial computed tomographic (CT) scan showing the hyperdense extra-axial mass located in the subdural and subgaleal space of the right parieto-occipital region. b Post-contrast CT scan reveals minimal homogeneous enhancement of the extra-axial mass lesion. c A bone-window CT image showing slight inner table irregularities in the right parieto-occipital region. Evliyaoğlu C et al. Primary Cranial Vault … Zentralbl Neurochir 2006; 67: 26 – 29 Downloaded by: WEST VIRGINIA UNIVERSITY. Copyrighted material. A 78-year-old woman was admitted to our hospital with progressive left hemiparesis, headache and a gradually increasing soft lump over the right parietal region 1 week after a head trauma due to falling from a high place. On examination, the patient had left hemiparesis and a palpable right parietal firm swelling, measuring 3 × 5 cm. The remainder of the examination was within normal limits, without any adenopathy or hepatosplenomegaly. Plain X-rays of the skull were assessed as normal. A computed tomography (CT) scan demonstrated a hyperdense parieto-occipital subdural and subgaleal lesion together with marked midline shift and white matter edema (Fig. 1 a). There was minimal homogeneous enhancement of the lesion after injection of intravenous contrast material (Fig. 1 b). The CT appearance suggested subdural-epidural hematoma. Considering her trauma story and clinical progression, the patient underwent an emergency operation with the diagnosis of subdural hematoma. However, a firm and grey-colored tumoros mass was found to lie beneath the galea, involving both skull and dura. The tumor was firmly attached to the dura and extended to the underlying brain parenchyma. The surgical approach was widened for a more extensive resection. The tumor was removed subtotally and a duraplasty was performed with a dura substitute. In the histopathological examination, the tumor cells were small lymphocytes with scant basophilic cytoplasm and clumped chromatin. Nucleoli were absent. Brain parenchyma and cranial bone marrow had a diffuse pattern of infiltration. There was also a variable proportion of large prolymphocytes. Pseudofollicles and regressive follicles were observed less commonly. Tumor cells expressed B cell associated antigens, LCA and CD20 were strongly positive (Figs. 2 a–c). CD5 and CD43 were observed to be weakly positive. CD10, bcl-2 CD15, CD30 and EMA were all negative. Staining for CD23 revealed a regressive meshwork of follicular dendritic cells. These findings led us to make the histopathological diagnosis of diffuse small B cell non-Hodgkin lymphoma in this case. After the operation, the patient was re-examined thoroughly. Peripheral lymph nodes, bone marrow biopsy, abdominal and thoracic CT were all normal. Laboratory findings were within normal limits. When the CT scan was re-examined, slight inner table irregularities (Fig. 1 c) were observed in the right parieto-occipital region, just beneath the mass lesion. Postoperative treatment consisted of whole brain irradiation with a total dose of 45 Gy in fractions and adjuvant chemotherapy (adriamycin, vincristine, cisplatin and prednisone). The patient has been followed up for four years and in this period her neurological examinations have been within normal limits. Case Report Case Report rietal lump, mimicking an organized subgaleal hematoma. In fact, the head trauma was only a surprising coincidence that may have augmented the patient’s symptoms. The management might have been altered into a more extensive surgical strategy by MRI which, if it had been done, would have excluded blood as the cause of the lesion. But unfortunately, an MRI study could not be obtained. Case Report b Skull lymphomas usually present as a mass lesion extending to both subcutaneous space and epidural space, and they may infiltrate meninges and/or cortex. Since primary cranial vault lymphoma predominantly afflicts the elderly population [5], it may be seen in such different clinical presentations. MRI studies can easily describe the meningeal and parenchymal invasion [2, 6]. Recent advances in MRI, including MR spectroscopy and diffusion-weighted imaging may even provide additional information in differential diagnosis. However, MRI is not widely used to image acute head injuries because of the time involved, the need to place the patient’s entire body into the bore of the magnet, and the potential difficulties posed by the ferromagnetic monitoring and resuscitative equipment used in the emergency rooms. On the other hand, destruction of the cortical bone may be less prominent as this tumor has a permeative growth pattern with a large soft tissue component [4], rendering the X-ray studies and CT scans unreliable. 28 c Fig. 2 a–c a Erosion and destruction of the osteoid tissue by neoplastic lymphoid cells (H&E, original magnification × 100). b Immunohistochemical characterization of malignant lymphoid cells in non-Hodgkin lymphoma. They show a strong expression of LCA (LCA immunostaining, original magnification × 400). c Lymphoid cells show also a strong expression of CD20 (CD20 immunostaining, original magnification × 200). Discussion In the emergency setting, CT still remains the most frequently used imaging modality for several reasons. It can detect almost all surgically significant lesions, costs significantly less than MRI, necessitates a shorter examination time, and is widely available. In our case a skull lymphoma was preoperatively misdiagnosed as a subdural hematoma considering the CT appearance, trauma story, and age of the patient; together with the paEvliyaoğlu C et al. Primary Cranial Vault … Zentralbl Neurochir 2006; 67: 26 – 29 The key to successful diagnosis and treatment is a high index of suspicion and complete diagnostic evaluation. Inner table irregularities of the parieto-occipital region on bone window CT studies or osteolytic lesions on direct X-ray studies might have suggested the possibility of other lesions beside the trauma; unless these findings had been overlooked. This case is another example where magnetic resonance imaging (MRI) is the ultimate and definitive test in the emergency ward whenever CT demonstrates any findings which are unclear. References 1 Agbi CB, Bannister CM, Turnbull IW. Primary cranial vault lymphoma mimicking a meningioma. Neurochirurgia (Stuttg) 1983; 26: 130 – 132 2 Aslan Y, Okten A, Demirci A. 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