Keratin 100% definitional ini1 expression retained 100%. Clinical features and mr imaging findings in 15 patients e. Classification of meningiomasadvances and controversies. It resembles other types of rhabdoid tumors with a great tendency for recurrence 1,2. We have studied an uncommon case of rhabdoid papillary meningioma in a 15yearold boy with a durabased mass arising in the left posterior fossa. Tumors involving the parotid are predominantly primary with metastatic lesions forminga miniscule population. She presented 4 years later in her 16th week of gestation with a large recurrence, subtotal resection of the tumor was performed, and the histopathology was consistant with rhabdoid meningioma6. Leptomeningeal dissemination of intraventricular rhabdoid.
Meningioma is perhaps the primary neuroepithelial tumor with the widest diversification in histologic pattern. Although the clinical and pathologic findings of rm have recently been well known,6,810 the radiologic features of rm have been rarely addressed in the literature. Primary calcified rhabdoid meningioma of the cranio. Recurrent skull base rhabdoid meningioma in an adolescent. The rhabdoid cells had eccentric round to oval nuclei, fine to coarse chromatin and prominent nucleoli, abundant eosinophilic cytoplasm tablefig2e. Leptomeningeal dissemination refers to diffuse seeding of the leptomeninges by tumor metastases. Histopathological diagnosis rhabdoid meningioma rm is an uncommon, aggressive variant of meningioma, designated as who grade iii malignancy. Rhabdoid meningioma is a rare subtype of meningiomas accounting for % of all intracranial meningiomas and classified as who grade iii tumor. Meningioma with rhabdoid morphology is associated with a poor prognosis, regardless of tumor hist. To the best of the authors knowledge, this is the first case of an ectopic orbital rhabdoid meningioma reported in the literature. Common sense holds that the risk of recurrence depends on the extent of removal. Rhabdoid morphology was defined as sheets of loosely cohesive cells with eccentric nuclei and hyaline, paranuclear inclusions. Malignant rhabdoid tumour mrt is a very aggressive form of tumour originally described as a variant of wilms tumour, which is primarily a kidney tumour that occurs mainly in children mrt was first described as a variant of wilms tumour of the kidney in 1978.
Olavs hospital, university hospital trondheim 680,110 habitants in 2011. We describe the cytopathologic findings on cerebrospinal fluid of one such case in a 26yrold female who underwent resection of a left temporoparietal mass. Morphologically, metastatic rhabdoid meningioma may mimic a primary or metastatic carcinoma, melanoma and sarcoma. Intermediate mitotic rate 4 mitoses10 hpf for whatever hpf means, see hpfitis. Neurosurgical care in midnorway, which includes three counties, is centralised at st. Rhabdoid meningioma is a rare and aggressive subtype of meningioma, it is classified as who grade iii. Adgebite and colleagues 7 found a 37% to 55% recurrence rate at 20 years and stafford and colleagues 3 quoted a 25% recurrence rate. Rhabdoid meningioma is a rarely seen subtype of meningioma, accounting for 1%3% of all intracranial meningiomas. Pdf rhabdoid meningioma lacking malignant features. Malignant rhabdoid tumour mrt is an aggressive sarcomatous neoplasm usually arising in the kidney, with a histology distinct from wilms tumour. Departments of pathology and 1neurosurgery, indira gandhi medical. Atypical meningioma by histomorphology either a or b. Subsequently, rhabdoid tumors and tumors with rhabdoid features have been described in many other. Feb 01, 2015 rhabdoid meningioma is an aggressive tumor, with propensity of remote metastasis and csf dissemination, and mimics metastatic carcinoma and atrt, microscopically.
The patient exhibited prominent extracranial extension during the past 6 years, consisting of a mixture of both perivascular pseudopapillary growth and rhabdoid cytologic features of neoplastic meningothelial cells. About 60% will be in the posterior cranial fossa particularly the cerebellum. Complete excision along with dural attachment, appropriate histological diagnosis and grading, and adjuvant radiotherapy are imperative for proper management. One of these cases was also studied by electron microscopy. Metastatic rhabdoid meningioma of the parotid mimicking. Although its recurrence is common, extracranial metastasis is rare and usually misdiagnosed. Ectopic rhabdoid meningioma of the orbit in a child.
Meningioma metastasizing to the parotid is extremely rare and hence can oftenbe mistaken for the more common primary salivary gland neoplasms. Meningothelial features included whorl formation and nuclear pseudoinclusions, immunohistochemical coexpression of vimentin and. Here we present a rare case of rhabdoid meningioma in a 24 year old lady with complaints of focal. Rhabdoid tumours outside the kidney were later reported in. The patient was started on a regimen of oral sunitinib and remained free of recurrence at 1. There were also pathological features consistent with oligoastrocytoma. Meningioma, the term coined by harvey cushing, refers to a set of tumors that arise contiguously to the meninges see the image below. Rm exhibits a striking histological resemblance to other rhabdoid tumors and strong. An atypical teratoid rhabdoid tumor atrt is a rare tumor usually diagnosed in childhood.
Rhabdoid meningioma arising concurrent in pulmonary and. Histopathology revealed a tumor composed entirely of rhabdoid cells in dyscohesive sheets tablefig2c with multiple, interspersed foci of necrosis tablefig2d. Calcified, cranio cervical junction, primary, rhabdoid meningioma introduction rhabdoid meningioma rm is a relatively new subtype of meningioma, first described in 1998 by kepes et al. It has an increased proliferative activity and is classified as a world health organization who grade iii meningioma 5. Rt involving the central nervous system cns is called atypical teratoid rhabdoid tumor. It is characterized by a high risk to local recurrences, neoplastic dissemination. Histopathology and mib1 labeling index predicted recurrence of meningiomas. Pdf rhabdoid meningiomaan uncommon and aggressive variant. The patient had a resection and the biopsy demonstrated a malignant meningioma with rhabdoid features, likely meningeal in origin who grade iii meningioma with rhabdoid morphology is associated with a poor prognosis, regardless of tumor histogenesis. The specimen had neither vascular proliferation usually seen in high. Rhabdoid meningioma rm is a recently described variant of malignant meningioma, with radiologic features currently not well characterized in the medical literature. Meningiomas demonstrate specific genetic and epigenetic mutations, whose effect on recurrence and progression is still of limited understan.
We emphasized the squash cytology and histology finding of the rare neoplasm. The operated patient with tentorial meningioma had radiotherapy due to presence of few mitosis in the tumor. Ultrastructurally, the latter consisted of whorls of intermediate filaments often entrapping lysosomes or other organelles. Frequencies of meningioma subtypes and histological malignancy grades have changed because of different classification systems. Of the 16 certs, only 2 a rhabdoid meningioma and a carcinoma with rhabdoid features. Pathological specimens revealed the diagnosis of rhabdoid meningioma. Treatment of meningioma typically consists of surgery alone, but radiation therapy can be used in cases of surgically inaccessible, residual, or recurrent tumors, as well as in patients with an unacceptably high surgical risk. Intraoperative diagnosis using smear preparation kirby, patricia a. The patient had a resection and the biopsy demonstrated a malignant meningioma with rhabdoid features, likely meningeal in origin who grade iii. Rhabdoid meningioma of brain a rare aggressive tumor ncbi. These tumors are associated with a poor prognosis and a high rate of recurrence 1, 2. A 59yearold male presented with a swelling in the left parotid region.
In most cases, the first symptoms are linked to the. Pdf primary pulmonary meningioma with rhabdoid features. Therefore, malignant brain tumors with globular inclusions, such as rhabdoid meningioma and atypical teratoid rhabdoid tumors should be considered in the differential diagnosis. They further suggest that rhabdoid meningiomas are highly aggressive tumors and that the rhabdoid phenotype represents a marker of malignant transformation in meningiomas. Transformation of grade ii to grade iii rhabdoidpapillary. Aggressive, rhabdoid meningioma, squash cytology, who grade iii.
Intraoperative touch cytology for an orbital tumour izu. Histologically, both tumors consisted of an admixture of undifferentiated polygonal or elongated cells and cells with abundant eosinophilic cytoplasm frequently containing hyaline globular structures. The purpose of this study was to describe and characterize clinical features and imaging findings associated with rm. In view of the history, neuroradiology, histopathology and immunohistochemistry, a final diagnosis of metastatic rhabdoidmeningioma to the parotid was rendered. It resembles other types of rhabdoid tumors with a great tendency for recurrence 1,2 extensive necrosis in rhabdoid meningioma might be indicative of an additional predictor. It has an aggressive course and needs to be treated by both surgery and radiotherapy. Rhabdoid meningioma rm is an uncommon, aggressive variant of meningioma, designated as who grade iii malignancy. The behavior of rhabdoid meningiomas otherwise lacking malignant features. All patients were explored ct, mri, preoperative selective angiography and in all cases the who 2000 classification criteria were used to define atypical meningioma results. Rhabdoid tumor genetic and rare diseases information. Therefore, malignant brain tumors with globular inclusions, such as rhabdoid meningioma and atypical teratoidrhabdoid tumors should be considered in the differential diagnosis. Histological classification and molecular genetics of meningiomas.
Histopathology revealed that the main component of the tumor was typical rhabdoid cells with some necrotic areas. Rhabdoid and papillary meningioma with leptomeningeal. Rhabdoid cells with abundant eosinophilic cytoplasm, cytoplasmic hyaline inclusions, eccentric nuclei stout processes consistent with meningioma. Ct n 8 and mr n 15 images of 15 patients 4 men and 11.
Anaplastic meningioma with cellular anaplasia and numerous mitotic figures a, rhabdoid. Malignant rhabdoid tumour mrt is a very aggressive form of tumour originally described as a variant of wilms tumour, which is primarily a kidney tumour that occurs mainly in children. N2 it is has been suggested that rhabdoid morphology is associated with a poor prognosis, regardless of tumor histogenesis. Rhabdoid transformation of tumor cells in meningiomas. Clinical features and mr imaging findings in 15 patients american journal of neuroradiology, vol. Clinical and histopathological features of two cases of malignant rhabdoid tumor of the kidney are presented. In the series of 15 cases by perry et al, patients had tumor recurrence and 8 died, with a median time to death of 3. Primary rhabdoid tumor with low grade glioma component of the. Rhabdoid meningioma rm was first described in 1998 as an unusual variant of meningiomas 5. Meningiomas account for up to 2430% of all primary intracranial neoplasms and majority have a benign course. Rhabdoid tumor rt is an aggressive pediatric soft tissue sarcoma that arises in the kidney, the liver, the peripheral nerves and all miscellaneous softparts throughout the body.
This difference was statistically significant p meningioma. Intraoperative diagnosis using smear preparation rhabdoid meningioma. It is characterized by a high risk to local recurrences, neoplastic dissemination and remote metastases. The dura is opened, and the meningioma can be seen extending en plaque over the surface of the brain.
The histology of rhabdoid meningioma resembles that of a primary atypical teratoidrhabdoid tumor of the. Primary rhabdoid tumor with low grade glioma component of. Excludes clear cell, chordoid, papillary, and rhabdoid subtypes. Histological variant other than clearcell, chordoid, papillary, or rhabdoid lacks criteria of atypical and anaplastic meningioma atypical meningioma who grade ii any of three criteria mitotic index. Seemingly complete removal is achieved in 64% to 97% of operated patients 1 5 but is curative only in 68% to 80%. A histologic indication of increased proliferative activity. Between 20062011 we operated 6 atypical meningioma of 63 benign meningiomas 9,52%. Rhabdoid meningioma was initially defined in 1998 in 2 case series by kepes et al and perry et al, who described a total of 19 patients with rhabdoid meningiomas.
The recurrence rate 20 years after seemingly radical surgery simpson grades 12 for benign meningiomas was at least 19% in a finnish populationbased study. Primary calcified rhabdoid meningioma of the craniocervical. Differential diagnosis extrarenal rhabdoid tumor surgical. Multiple extracranial metastases from a rhabdoid meningioma bvs. The full text of this article is available in pdf format. This difference was statistically significant p mar 11, 2005 rhabdoid cells are encountered in specific entities, such as malignant rhabdoid tumor and atypical teratoid rhabdoid tumor, as well as in composite rhabdoid tumors derived secondarily from other. Mrt was first described as a variant of wilms tumour of the kidney in 1978. Muscle markers negative definitional keratin 550% positive, focal, weak. Rhabdoid meningioma rm is a rare, aggressive variant of meningioma classified as a who grade iii malignancy. Rhabdoid meningioma is an extremely rare subtype of who grade 3 meningiomas characterized by the presence of cells resembling rhabdomyoblasts and exhibiting vimentin immunoreactivity. It resembles other types of rhabdoid tumors with a great tendency for recurrence 1,2 extensive necrosis in rhabdoid meningioma might be indicative of an additional predictor of aggressive clinical. Mrts are a rare and highly malignant childhood neoplasm. Malignant meniongiomas including papillary meningioma, rhabdoid meningioma and anaplastic meningioma account for less than 5 percent of all meningiomas.
Granulofilamentous meningioma is a rare variant of benign meningioma characterized by eosinophilic intracytoplasmic globular inclusions on cytopathology and histopathology. Some of these patterns may suggest a diagnosis other than meningioma, particularly when the biopsy sample is small and during intraoperative consultation. Recurrence of meningiomas and its management clinical gate. Although usually a brain tumor, atrt can occur anywhere in the central nervous system cns, including the spinal cord. Rhabdoid meningioma is a rarely seen subtype of meningioma, accounting for 1%3% of all. Meningiomas with rhabdoid features lacking other histologic. Rhabdoid morphology is associated with a poor prognosis, regardless of tumor histogenesis. Pdf we reported a case of meningioma with rhabdoid morphology but lacking. Multiple extracranial metastases from a rhabdoid meningioma mesquita filho et al. Rhabdoid meningioma is a recently described, rare, who grade iii intracranial tumor with an aggressive growth pattern and increased risk of recurrence. Pdf only 1% to 2% of meningiomas have primary extrameningeal. Rhabdoid meningioma rm is an unusual variant of meningioma, classified as world health organization grade iii. The histopathological spectrum of human meningiomas. Herein, we report 2 additional cases of rhabdoid meningiomas with no histological evidence of.
Rhabdoid meningioma is an aggressive tumor, with propensity of remote metastasis and csf dissemination, and mimics metastatic carcinoma and atrt, microscopically. It is a named variant of anaplastic meningioma and belongs to who grade iii category. Rhabdoid meningioma is derived from the arachnoidal epithelial cells and contains cells resembling rhabdomyoblasts, i. For benign meningiomas, clinically relevant recurrences are common during the patients lifetimes. Oct 14, 2015 dural tail sign has high specificity but low sensitivity to detect intracranial meningioma level 1 likely reliable evidencebased on diagnostic cohort study 110 patients aged 680 years with an intracranial mass were evaluated with mri with and without contrast followed by resection and histopathology of mass reference standard 98. Histologically, both tumors consisted of an admixture of undifferentiated polygonal or elongated cells and cells with abundant eosinophilic cytoplasm frequently containing hyaline globular. All patients treated for a primary meningioma over a ten year period, from 1. Ugnius mickys at national centre of pathology, vilnius university. This case report shows imaging findings and differential diagnosis of leptomeningeal dissemination of intraventricular rm.
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