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