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