Subdural sarcoma associated with chronic subdural hematoma

7 downloads 78 Views 894KB Size Report
lection and the subsequent development of a subdural sarcoma. KEY WORDS. • liposarcoma • fibrosarcoma • subdural hematoma • pericerebral collection ...
J Neurosurg 86:553–557, 1997

Subdural sarcoma associated with chronic subdural hematoma Report of two cases and review of the literature GIUSEPPE CINALLI, M.D., MICHEL ZERAH, M.D., MICHEL CARTERET, M.D., FRANÇOIS DOZ, M.D., LAURENT VINIKOFF, M.D., ARIELLE LELLOUCH-TUBIANA, M.D., BEATRICE HUSSON, M.D., AND ALAIN PIERRE-KAHN, M.D. Departments of Pediatric Neurosurgery, Pediatric Radiology, and Anatomic Pathology, Hôpital Necker-Enfants Malades, Université René Descartes; Department of Pediatric Oncology, Institut Curie, and Department of Pediatric Radiology, Le Kremlin Bicêtre Hospital, Université Paris Sud, Paris, France U The authors report the cases of two children who presented in the first months of life with progressive macrocrania related to chronic pericerebral fluid collection. This condition resolved spontaneously without treatment after a few months in the first case, whereas it required several aspirations of blood-stained fluid via the fontanel in the second case. Both patients developed normally without evidence of disease in the earliest years of life and presented at the ages of 3 1/2 and 4 1/2 years, respectively, with symptoms and signs of rapidly progressing intracranial hypertension. In both cases contrast-enhanced computerized tomography and magnetic resonance imaging revealed masses in the subdural space of the skull base and the cranial vault associated with significant subdural fluid collections. In the first case the lesion was misdiagnosed in the initial phase and treated, by means of multiple craniotomies, as an organized subdural hematoma. After a diagnosis of liposarcoma had been made, the patient was treated with chemotherapy, which resulted in a good resolution of the lesions at 3-month follow-up review. In the second case a biopsy allowed the diagnosis of fibrohistiocytic sarcoma and the patient was treated with chemotherapy. The authors review the literature of the few reported cases and discuss the possible pathophysiological association between pericerebral fluid collection and the subsequent development of a subdural sarcoma.

KEY WORDS • liposarcoma • fibrosarcoma • subdural hematoma • pericerebral collection • intracranial neoplasm • chemotherapy

HE association between subdural hematoma and subdural sarcoma has already been reported.1,3,4,8,11 In some cases1,3,11 the subdural effusion was diagnosed at the same time as the neoplasm, whereas in other cases, including the two reported here, the sarcoma developed in the cavity of a subdural hematoma that had been diagnosed or treated several years before.1,4,8 In the first cases the subdural effusion seemed to be a secondary manifestation of a primary subdural tumor and was probably induced by tumor secretion or chronic subdural bleeding originating from the tumor. In the other cases, the neoplasm could be considered a late complication of the primary sudural hematoma, because this condition was diagnosed and treated several years before the onset of symptoms and signs related to the subdural sarcoma. We report these cases because of their rarity, the possible diagnostic error and dangerous delay in appropriate treatment that can be induced by the previous clinical history and because these are the first cases studied using magnetic resonance (MR) imaging.

T

J. Neurosurg. / Volume 86 / March, 1997

Case Reports Case 1 History. This 6-month-old girl was admitted to the pediatric department of another hospital on September 15, 1992 because of partial seizures. She had a history of mild head trauma due to a fall from a height of 50 cm 15 days before. The computerized tomography (CT) scan obtained at admission revealed signs of recent subdural bleeding with a mild pericerebral collection. Three days later she experienced three seizure episodes, after which a repeated CT scan revealed a bilateral asymmetric pericerebral fluid collection, and the fontanel became bulging. Several subdural taps through both angles of the fontanel were performed, allowing the aspiration of more than 300 ml of hematic fluid. The patient was discharged on November 5, 1992. She was followed in outpatient clinics in subsequent months and a progressive increase in head circumference was observed (6 5 standard deviations). An MR image 553

G. Cinalli, et al.

FIG. 1. Case 1. Left: Axial T1-weighted MR image (TR 600 msec/TE 20 msec) with gadolinium enhancement, showing bilateral frontal chronic subdural collections isointense to cerebrospinal fluid. Recent bleeding in the right subdural cavity reveals loculation within the pericerebral fluid collection. Note the moderate mass effect on frontal lobes and right ventricle. Center: Axial T1-weighted MR image (TR 600 msec/TE 20 msec) with gadolinium enhancement, showing meningeal thickening and nodular enhancements. Note the recent hemorrhage on the left side (methemoglobin), and the important mass effect on the frontal lobes. Right: Axial noncontrast-enhanced CT scan obtained 3 months after placement of a subdural peritoneal shunt. A heterogeneous subdural mass on the right side shifts the midline, with important mass effect on the ventricles.

obtained on February 4, 1993 revealed a stable pericerebral collection with evidence of recent bleeding (Fig. 1 left). The patient developed normally, with acquisition of developmental milestones at normal intervals. Examination and Operation. On June 8, 1995 the patient was admitted for the first time to our department of pediatric neurosurgery with a 2-week history of vomiting and decreased consciousness. An MR image obtained on admission showed an asymmetric subdural fluid collection with several enhancing nodules bulging in the subdural cavity (Fig. 1 center) and a subdural peritoneal shunt was inserted 10 days later. The fluid sampled at surgery was noted to be yellowish and unusually thick. The postoperative CT scan revealed an almost complete resolution of the right frontal collection with a residual cavity filled by a hypodense tissue that was interpreted as fibrous organization of the residual cavity. Repeated Operations, Chemotherapy, and Outcome. The patient was admitted to our department 3 months later with a 1-week history of headache and vomiting. A CT scan revealed a huge right frontoparietal lesion with midline deviation associated with a right frontobasal and a right parietal cyst (Fig. 1 right). The patient underwent craniotomies on September 8, September 12, and October 5 for the removal of the parietal, frontal, and posteroparietal components of the lesion, which appeared at surgery as thick, grayish, vascularized fibrous tissue that was adherent to the inner and outer membrane of the subdural cavity. Several noncontrast-enhanced CT scans revealed a progressive increase of the residual components of the lesion at the level of the cranial base and a rapid regrowth of the previously removed lesion. Clinically, the patient rapidly deteriorated, with symptoms including loss of gait, left hemiparesis, visual dysfunction, mutism, and drowsiness. A contrast-enhanced CT scan revealed a strong enhancement of the subdural mass, which was later con554

firmed by MR imaging (Fig. 2 left and center). These features prompted us to ask for a review of the histology. In fact, fat staining resulted in the diagnosis of liposarcoma. Because of the rapidity of tumor regrowth and the clinical deterioration, the patient underwent repeated surgery on November 7, 1995. All the bone flaps were removed and all the visible tumor on the convexity was resected. The patient was then transferred to the Pediatric Department of the Institut Curie where chemotherapy consisting of ifosfamide, carboplatin, and etoposide was initiated.6 After two courses of chemotherapy, the patient rapidly improved and MR imaging demonstrated a significant reduction in the size of the frontobasal mass and of the residual tumor on the convexity and in the interhemispheric fissure (Fig. 2 right). In May of 1996 the lesion recurred in the parietal region and rapidly increased both intracranially and subcutaneously. The parents refused further treatment and the patient died on September 18, 1996. Histological Findings. Large tumor cells were clearly visible on smears, with large cytoplasmic vacuoles of different sizes in every cell. These vacuoles stained positive for fat with Oil Red O (Fig. 3). Anisokaryosis and mitoses were common. The same findings were observed with paraffin-included stains, with frequent mitoses and large vessels with thin walls. The tumor invaded the dura, bone, and soft tissues. Vascular markers (HY, CD34, CD31), as well as glial markers (GFA), were negative on immunohistochemical studies, and vimentin was positive. Case 2 History and Examination. This 4 1/2-year-old boy was referred to our department on January 31, 1996. He had been born prematurely at 32 weeks of gestational age. At the age of 4 months he presented with a progressive macrocranium and cranial ultrasonography revealed a biJ. Neurosurg. / Volume 86 / March, 1997

Meningeal sarcoma and subdural hematoma

FIG. 2. Case 1. Left and Center: Before chemotherapy. Sagittal (left) and axial (center) spoiled gradient-recalled MR images with gadolinium enhancement, showing a diffuse meningeal tumoral extension under the frontal lobes, posterior interhemispheric region, and parietal convexity with a deep invagination displacing the brain structures. Right: A neurological study obtained after chemotherapy. Axial T1-weighted MR image (TR 600 msec/TE 20 msec) showing meningeal enhancement. Subdural spaces are quite normal except in the right parietal and interhemispheric regions. No more mass effect is seen on the ventricles.

lateral pericerebral fluid collection. Ultrasonography was repeated 2 months later and showed the stability of the collection. The patient was not treated and his head circumference evolution normalized in a few months. In the last 6 months the patient had been complaining of headache, and repeated vomiting had appeared in the last 3 days. Funduscopic examination revealed right papilledema and normal left fundus. The CT and MR studies demonstrated a huge right frontotemporal contrast-enhancing mass associated with a bilateral frontoparietal subdural fluid collection. The walls of the subdural collection enhanced after injection of contrast material and displayed several enhancing nodules on both the cortical and the cranial layers (Fig. 4). Operation and Postoperative Course. The patient underwent operation on the same day: a right frontal craniotomy was performed with a trephine. At the opening of the dura a thick yellow membrane was encountered, the incision of which allowed the drainage of a huge amount of yellow fluid. This membrane covered both the cortical and cranial surface of the subdural cavity and contained a gray–yellow nodule from which a biopsy specimen was obtained. An external subdural drain was left in the cavity and withdrawn the following day. Cytological examination of the subdural fluid revealed signs of chronic inflammatory reaction and foamy macrophages filled with fatty nodules. Histological examination of the mural nodule revealed a fibrohistiocytic sarcoma. The postoperative period was uneventful and the patient was sent to the pediatric department of the Institut Curie for chemotherapy according to the same protocol as in Case 1.6 Histological Findings. The cytological composition of the subdural fluid revealed red blood cells and large macrophages with cytoplasmic vacuoles positive for Oil Red O staining. The outer wall of the subdural collection was thick and dense with collagen fibers, with a proliferation of fibrohistiocytic cells on its inner aspect. The tumor nodule displayed a collagenous hyaline core with many vessels and a peripheral area with a proliferation of fibrohistiocytic cells with irregular, multiple nuclei. The cytoplasm was large, with large vacuoles. The cells were J. Neurosurg. / Volume 86 / March, 1997

surrounded by abundant collagen fibers. Immunohistochemical studies showed that vimentin, KP1, and MIB-1 were strongly positive, and S100 protein was negative. Discussion Subdural sarcomas are an extremely rare entity. In an attempt to classify the sarcomas of the meninges, Bailey and Ingraham1 said that “a sharp distinction should be drawn between the sarcomas of the leptomeninges and the sarcomas of the dura,” the former spreading freely in the subarachnoid spaces, the latter being limited to the dura and the subdural space without penetration of the arachnoid layer. The association of this type of subdural sarco-

FIG. 3. Case 1. Oil Red O smear. Liposarcoma. Note the large cytoplasmic vacuoles in the neoplastic cells. Original magnification 3 250.

555

G. Cinalli, et al.

FIG. 4. Case 2. Sagittal T1- (left) and axial T2-weighted (right) MR images obtained after injection of contrast enhancement, showing the frontal solid mass and enhancing nodules bulging in the cavity of the anterior and posterior pericerebral fluid collection.

ma with chronic subdural hematoma is even more rare. In their series of three cases of fibrosarcoma of the dura, these authors reported two cases of primary subdural sarcoma in children which featured bilateral involvement of the subdural spaces, mimicking a subdural hematoma. In the first case the patient had a history of progressive macrocranium and bulging fontanel from the age of 3 months. This condition was left untreated. At the age of 4 months the patient remained stuporous for 2 days after a fall. He was well until the age of 5 1/2 years, when he was admitted for drowsiness and gait disturbances, with skull masses in the right parietal and temporal regions. A subdural tap allowed the aspiration of brown, semigelatinous fluid, and two craniotomies allowed the partial excision of a large subdural tumor that was found to be a fibrosarcoma. Radiation therapy resulted in the complete regression of the tumor, but it recurred a few weeks later and the patient died several weeks after clinical presentation. The second case was a newborn infant who presented with an episode of cyanosis at 11 days of age, accompanied by a bulging fontanel and increasing head circumference. At 27 days a subdural tap allowed the aspiration of thick brown fluid and a small craniotomy allowed the biopsy of a subdural malignancy that was revealed to be a fibrosarcoma. Other cases of subdural sarcoma associated with subdural hematoma have been reported. In 1970, Kothandaram4 described the case of a girl who presented at the age of 4 months with a history of increasing head circumference and vomiting. Subdural hematoma was diagnosed by aspiration of blood-stained fluid via the fontanel. The patient was treated by repeated subdural taps, burr hole placement, and drainage and was discharged 3 weeks after admission with a concave fontanel. She was readmitted 2 years and 3 months later because of headache, vomiting, increasing drowsiness, and bilateral papilledema. A burr hole revealed that the subdural space was filled with brown saucelike material and a nodular layer of thick white material on the floor of the subdural cavity. The patient deteriorated progressively and died 1 month later. Postmortem examination showed a tumor filling the subdural space. The histological diagnosis was liposarcoma. 556

In 1973, Kostka and Drahozal3 described the case of a 21-year-old patient who died 6 months after presentation with a subdural sarcoma that was associated with a subdural fluid collection. At the age of 11 years the patient had been admitted to the hospital for head trauma that was complicated by bilateral papilledema. No CT scan was obtained. Nussbaum, et al.,8 reported the case of a 76-year-old man who had been treated for a subdural hematoma by means of burr holes 15 years earlier. At the time of presentation for neurological deterioration, his CT scan revealed a heterogeneous hyperdense region over the left frontoparietal convexity that was interpreted as an acute hemorrhage into the old subdural hematoma. Surgery allowed the partial removal of a meningeal-based sarcoma. The analysis of these cases seems to show that subdural sarcomas associated with pericerebral fluid collections have two modalities of onset. In the first scenario, as in our two cases, the pericerebral collection is diagnosed and treated several years before the clinical and radiological diagnosis of the malignancy.1,3,4,8 The pathophysiology of the pericerebral collection is variable, ranging from posttraumatic chronic subdural hematoma in the elderly8 to benign, self-resolving pericerebral collection in the newborn (our Case 2) and posttraumatic blood-stained subdural effusion in the newborn (our Case 1). In these cases, the pericerebral fluid collection seems to be the primary pathology, without clinical or radiological evidence of malignancy at initial diagnosis and treatment. This fact appears to be confirmed by the very long delay between the treatment of the pericerebral fluid collection and the early clinical manifestations and radiological evidence of the neoplastic growth (3–15 years). The absence of malignancy at this stage of the disease’s evolution is clear in our Case 1, in which follow-up contrast-enhanced MR imaging performed several months after the treatment and stabilization of the pericerebral collection did not reveal any pathological contrast enhancement. Nussbaum, et al.,8 cited anecdotal reports of tumors arising at the site of previous surgery5,9,12 and proposed that a direct correlation could exist between the traumatic event and the later onset of the tumor. In a recent review of soft-tissue sarcomas arising in the pleural cavity, Myoui, et al.,7 found that 47% of these patients had a history of chronic pleural inflammatory disease, including tuberculous pleuritis or pyothorax, pleuritis of unknown cause, and pulmonary tuberculosis. In a review of the literature, these authors found several cases of pleural malignancy arising in the setting of pleural inflammatory disease, suggesting that “long standing pleural inflammation might be an etiologic factor for development of soft tissue sarcoma.” The analysis of the literature suggests that a similar mechanism could be responsible for the neoplastic growth at the site of chronic pericerebral fluid collection. The inflammatory reaction usually found in chronic subdural hematoma associated with the proliferative growth related to the formation of the membrane of the cavity could be the etiological factor for development of subdural sarcoma. In some cases surgical trauma or repeated taps could amplify the risk, although this would be an extremely rare event if we consider the high frequency of subdural hematomas, both in children and in the elderly. The second pattern of presentation differs in the abJ. Neurosurg. / Volume 86 / March, 1997

Meningeal sarcoma and subdural hematoma sence of clinical history of pericerebral fluid collection or progressive macrocranium.1,4,11 The subdural fluid effusion is discovered at surgery or by fontanel tapping at the time of the diagnosis of the subdural malignancy, and it should be interpreted in these cases as the product of tumor secretion or chronic bleeding from tumor vessels in the subdural space. Subdural Liposarcoma Intracranial liposarcomas are an extremely rare entity. To the best of our knowledge, only two cases have been reported so far,4,10 both arising in the meninges of the cranial vault. The case described by Sima, et al.,10 was that of a 70-year-old woman with no medical history who suddenly developed gait and speech disturbances. A tumor of the left frontal convexity was discovered by arteriography, and surgery allowed the removal of a liposarcoma attached to the inner aspect of the dura in the absence of pericerebral fluid collection. In contrast, the similarities between the case described by Kothandaram4 in 1970 and the one reported here are obvious. Both patients presented in the neonatal period with what seemed to be a common subdural fluid collection of infancy and were treated by repeated subdural tapping. They both presented again after a disease-free interval of more than 2 years with symptoms and signs of a rapidly growing liposarcoma developing in the cavity of the old subdural hematoma. The pericerebral fluid collections could be interpreted as a very early manifestation of a primary subdural tumor, but several considerations weigh against this hypothesis. In our case, the pericerebral collection was likely to be the consequence of the head trauma that had occurred 15 days before. The CT and MR images obtained initially and in the following years failed to show any tumor growth until the admission to our department, when MR imaging revealed contrast-enhancing nodules on the inner and outer membrane of the subdural cavity. In less than 2 months from the time they were noted, these nodules grew very rapidly to form a huge mass. It is unlikely that such a rapidly growing tumor could exist at the time of the diagnosis of the pericerebral collection and remain silent for more than 2 and 3 years, respectively. The history of chronic pericerebral fluid collection led to a very significant delay in establishing the correct diagnosis. The contrast-enhancing nodules revealed by MR imaging at the first presentation in our department were misinterpreted as nodular thickening of the subdural membrane, as can be observed in organized chronic subdural hematomas.2 When the patient presented 2 months later with a huge subdural mass, this was again misinter-

J. Neurosurg. / Volume 86 / March, 1997

preted has having been caused by rebleeding in the cavity of the old hematoma, contrast enhancement was not used for CT scans after each craniotomy, and pathological examinations favored organized hematoma. Contrast enhancement was used only after the infectious complication. After observing the strong contrast enhancement and the rapid regrowth of the mass, we asked for a review of the histological studies, which allowed the correct pathological diagnosis to be made with the use of fat stains. Acknowledgments We gratefully acknowledge Dr. Paul Dominic Chumas for his opinions and his help in reviewing the manuscript and Mr. Xavier Ferreira for technical assistance. References 1. Bailey OT, Ingraham FD: Intracranial fibrosarcomas of the dura mater in childhood: pathological characteristics and surgical management. J Neurosurg 2:1–15, 1945 2. Fujioka M, Okuchi K, Miyamoto S, et al: Bilateral organized chronic subdural haematomas: high field magnetic resonance images and histological considerations. Acta Neurochir 131: 265–269, 1994 3. Kostka Vl, Drahozal H: Méneˇ obvykly´ prˇípad sarkomatózy turdé pleny vytvárˇésící obraz subdurálního hygromu. Cesk Neurol 36:43–46, 1973 4. Kothandaram P: Dural liposarcoma associated with subdural hematoma. Case report. J Neurosurg 33:85–87, 1970 5. Kristoferitsch W, Jellinger K: Multifocal spinal angiosarcoma after chordotomy. Acta Neurochir 79:145–153, 1986 6. Marina NM, Rodman J, Shema SJ, et al: Phase I study of escalating targeted doses of carboplatin combined with ifosfamide and etoposide in children with relapsed solid tumor. J Clin Oncol 11:554–560, 1993 7. Myoui A, Aozasa K, Iuchi K, et al: Soft tissue sarcoma of the pleural cavity. Cancer 68:1550–1554, 1991 8. Nussbaum ES, Wen DYK, Latchaw RE, et al: Meningeal sarcoma mimicking an acute subdural hematoma on CT. J Comput Assist Tomogr 19:643–645, 1995 9. Russell DS, Rubinstein LJ: Pathology of Tumours of the Nervous System, ed 5. Baltimore: Williams & Wilkins, 1989 10. Sima A, Kindblom LG, Pellettieri L: Liposarcoma of the meninges. Acta Pathol Microbiol Scand A 84:306–310, 1976 11. Tzonos T, Kraus B: Subduraler Erguss als Folge eines diffusen Leptomeningealsarkoms. Neurochirurgia 6:227–231, 1972 12. Zülch KJ: Brain Tumors. Their Biology and Pathology, ed 3. Berlin: Springer-Verlag, 1986 Manuscript received June 24, 1996. Accepted in final form October 31, 1996. Address reprint requests to: Giuseppe Cinalli, M.D., Service de Neurochirurgie, Hôpital Necker-Enfants Malades, 149 rue de Sévres, 75015 Paris, France.

557