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Tumor mass of the cerebellopontine angle in a 28 years-old man

Giorgio Gherardi y Francesca Sparacio

Servizio di Anatomia e Istologia Patologica
Ospedale Fatebenefratelli e Oftalmico
Milano

Italia
Comentado en:
PATOLOGIA
PATOCITO
FOROPAT
 Historia Clínica
In the last two years the patient has experienced several attacks of cervico-occipital pain associated with nausea and vomitus; he was admitted to the hospital because of mental confusion and deep drowsiness developed after a recent attack. CT-scan shows that the tumor mass is causing compression of the fourth ventricle and supratentorial hydrocephalus. Upon surgery the tumor appears diffusely hemorrhagic and necrotic and only a small portion is resected for diagnostic purpose.
Imagen de Tumor mass of the cerebellopontine angle in a 28 years-old man
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CT-Scan.
Imagen de Tumor mass of the cerebellopontine angle in a 28 years-old man
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CT-Scan.
 Iconografía
Imagen de Tumor mass of the cerebellopontine angle in a 28 years-old man
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Imagen de Tumor mass of the cerebellopontine angle in a 28 years-old man
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Imagen de Tumor mass of the cerebellopontine angle in a 28 years-old man
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Imagen de Tumor mass of the cerebellopontine angle in a 28 years-old man
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Imagen de Tumor mass of the cerebellopontine angle in a 28 years-old man
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Imagen de Tumor mass of the cerebellopontine angle in a 28 years-old man
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 Comentarios

 

El 4/11/2007 20:19, Alonso dijo:

Oligodendroglioma?

Un cordial saludo.

 

El 4/11/2007 23:13, David Cubero dijo:

Podría ser un meningioma de células claras, indicaría un PAS para evidenciar glucogeno, EMA y otra IHQ para el diagnostico diferencial que puede ser extenso.

Saludos David

 

El 5/11/2007 0:33, Romualdo Correia Lins Filho dijo:

Dear Drs. Giorgio and Francesca

In my opinion this is a malignant neoplasm. I think the presence of some large eosinophilic nucleoli, the excentric eosinophilic cytoplasm of some epithelioid calls, and principally, the fine brown cytoplasmic granularity (figure 7) favor the diagnosis of MELANOMA, possibly primary, according to the prolonged duration of symptoms.

Best regards

Romualdo

 

El 5/11/2007 14:39, Hernan Molina Kirsch dijo:

Atypical teratoid/rhabdoid tumor.

Diferential diagnosis of CNS germinoma.

Beatifull and rare case.

Muy agradecido por presentarlo.

From near the Pacaya volcano.

Hernan

 

El 5/11/2007 15:08, Jorge Mogollon dijo:

Tumor maligno con rasgos rabdoides. No descarto la metástasis de un melanoma. Gracias

 

El 6/11/2007 4:45, Reynaldo Falcón Escobedo dijo:

This is classical example of Atypical teratoid/rhabdoid tumor

 

El 6/11/2007 12:13, Luis Briones dijo:

tumore atipico teratoide/rabdoide (ATRT)

 

El 7/11/2007 3:03, Victor Leonel Argueta Sandoval dijo:

Tumor Rabdoide. Considerarse los diagnóstico diferenciales de cuadros rabdoides de otros tumores, tal como gliomas y meningiomas, ante todo por edad de paciente.

 

El 7/11/2007 4:25, David Cubero dijo:

Coincido con el dgtco morfológico de t. Rabdoide / Teratoide.En contra:

Edad, evolución pre operatoria 2 años para una lesión grado 4, localizado cerebelo (adulto.

Ciertamente difícil.

Saludos David

 

El 7/11/2007 13:43, Miguel Reyes-Múgica dijo:

Tumor rabdoide/teratoide atípico, es una magnífica posibilidad. El estudio citogenéteico, con deleción o alteraciones del cromosoma 22(q11.2), o inmunohistoquímico con pérdida de la expresión de hSNF5/INI1 deben ser de gran utilidad para confirmar o descartar esa entidad.

 

El 8/11/2007 12:26, Giorgio Gherardi dijo:

This is an update to the case: immunostains for Actin, Desmin, EMA, GFAP, PlAP, S100P, HMB-45, CD57 gave negative results, while immunostain for Cytokeratin 8,18 disclosed the reactivity seen in Fig. 8. I wish also to point out that according to the radiologist reporting the CT-scan this is most probably an "extracerebral mass".

Ciao a tutti

 

El 9/11/2007 3:29, Hernan Molina Kirsch dijo:

I would like to know the neurosurgeon opinion about the tumor location. On the images it looks attached to the dura but there is more parenchimal infiltrate.

What about the results on Vimentin, CD99 and INI1?

Me gustaría conocer la opinión del neurocirujano acerca de la localización del tumor. En las imágenes da la impresión de que se encuentra adherido la dura pero existe marcad infiltración del parenquima cerebeloso.

¿Tiene resultados de la Vimentina,CD99 y el INI1?

Haberler C y cols.

Immunohistochemical analysis of INI1 protein in malignant pediatric CNS tumors: Lack of INI1 in atypical teratoid/rhabdoid tumors and in a fraction of primitive neuroectodermal tumors without rhabdoid phenotype.

Am J Surg Pathol. 2006 Nov;30(11):1462-8.

Perry A y cols.

INI1 expression is retained in composite rhabdoid tumors, including rhabdoid meningiomas.

The majority of malignant rhabdoid tumors and atypical teratoid/rhabdoid tumors have a polyphenotypic

immunoprofile that includes epithelial membrane antigen (EMA), vimentin, smooth muscle actin, and

CD99 positivity, as well as variable immunoreactivities for cytokeratins, glial fibrillary acidic protein

(GFAP), synaptophysin, neurofilament, S-100 protein, and desmin. In contrast, most composite

rhabdoid tumors retain the same or a slightly altered immunoprofile of the parent neoplasm.

Mod Pathol. 2005 Jul;18(7):951-8.

Desde este terruño en mesoamerica.

Hernan

 

El 10/11/2007 12:33, Alyssandra Almeida dijo:

Apesar da aparente ausência de focos cartilaginosos (que às vêzes são escassos e difíceis de demonstrar) acho que pode ser um condrossarcoma mesenquimal, pelos seguintes motivos:

1.A idade é compatível

2.Condrossarcomas mesenquimais frequentemente ocorrem em meninges

3.Há um padrão hemangiopericítico, melhor visto nas Figuras 3 e 4

4.Casos intracranianos podem ser positivos para citoqueratinas.

Abraços

Alyssandra

 

El 10/11/2007 14:13, Hernán Molina Kirsch dijo:

Just to be clear Mesenchymal Chondrosarcoma is extremely rare as primary or not primary meningeal tumor a fact easily corroborated with literature search through pubmed.

In my opinion there is a vascular component but it is not even remotely hemangiopericytic.

Es importante dejar en claro que el condrosarcoma mesenquimal es extremadamente raro como primario o involucrando a las meniges, puntual que se puede corroborar fácilmente con una revisión de literatura en pubmed.

En mi opinión el componente vascular presente no presenta caracteristicas hemangiopericitoides.

Saludes desde este promontorio volcánico del medio de la América.

Hernán Molina Kirsch

 

El 10/11/2007 18:20, Alyssandra Raulino dijo:

As meninges são, sim, classicamente referidas como uma das principais localizações do condrossarcoma mesenquimal:

1."Mesenchymal chondrosarcoma has a predilection for the meninges, orbit, neck, spine, and the lower extremities, although it has been reported in a wide variety of other sites". Kempson RL et al. Tumors of Soft Tissues, AFIP Atlas of Tumor Pathology, Series 3, page 405

2."Although it commonly has its origin in bone or soft tissue, it may arise in the meninges to produce a discrete mass that can compress brain and invade bone". Burger PC, Vogel FS. Surgical pathology of the nervous system and its coverings, ed. 2, 1982, Wiley Medical, page 131

3."Aproximately one-fifth to one-third of the lesions primarily affect the somatic tissssues and the meninges are one of the most common sites of extraskeletal involvement". Fletcher CDM et al. Pathology and Genetics . Tumors of Soft Tissue and Bone, 2002, IARC Press, page 255

4."This rare highly aggressive variant of chondrosarcoma may impinge upon the nervous system from a primary locus in the surrounding skull or spine, or arise directly from the dura or within brain parenchyma". Burger PC and Scheithauer BW. Tumors of the central nervous system. AFIP Atlas of Tumor pathology, Series 3, page 306

5."A high percentage of these neoplasms involve extraosseous structures, such as the orbit, paraspinal region, meninges or soft tissues of the extremities". Rosai and Ackerman's Surgical Pathology, ed 9, 2004, Mosby, page 2169.

Acredito que a possibilidade de um tumor rabdóide/ teratóide atípico seja extremamente improvável. A ocorrência em adultos é excepcional. Quase todos os casos ocorrem em crianças pequenas e apenas dois casos adultos haviam sido descritos à época da publicação da penúltima edição do fascículo da OMS sobre tumores do sistema nervoso central. No mesmo livro lê-se que apenas 10% dos tumores rabdóides/ teratóides atípicos ocorrem na região do ângulo cerebelo-pontino e que as células neoplásicas rabdóides quase sempre expressam EMA e actina. Esses marcadores foram negativos no presente caso.

 

El 12/11/2007 2:08, Reynaldo falcón Escobedo dijo:

Felicitaciones a los autores. Es un hermosísimo caso, con alto grado de doficultad,incluso en los tejidos blandos, mucho más en una localización intracraneal. El diagnóstico con la inmunohistoquímica y la demostración de la traslocación (X;18)(p11;q11)es claro.

Congratulations to the authors. This es a beautiful case with a high grade of difficulty even in soft tissue and of course. The diagnosis is clear with the immunohistochemistry and the translocation t(X;18)(p11;q11).

 

El 12/11/2007 3:50, Oscar Marin dijo:

Extraordinario caso. Felicitaciones y gracias por compartirlo.
Hacer un comentario a este caso
 Diagnóstico
POORLY DIFFERENTIATED SYNOVIAL SARCOMA.
 Comentario del Autor
It is well recognized that about 20% of synovial sarcoma cases belong to the “poorly differentiated” variant, which is characterized by high cellularity, with areas of necrosis and increased mitotic activity, and cells that can be small and round, but also larger and epithelioid, and can appear rhabdoid 1 . These tumors show about the same immunohistochemical pattern as usual types of monophasic synovial sarcoma, with the exception of EMA expression (which may be lacking in about 10-20% of cases) and the expression of S100 protein (which may be detected in up to 25% of cases) 2 , 3 . The tumors classified under this heading bear the translocation t(X;18) which seems specific for synovial sarcoma 2 , 3 . In the case under discussion, other than a peculiar expression of CK8,18 in about 30 to 40% of cells Figura_8 , we detected the expression of Vimentin, CD99, bcl-2 and calretinin as seen in Figura_9 . In addition, there was an occasional positivity for CD117(c-kit)(not illustrated). Other immunostains specific for S100P, HMB-45, EMA, Synaptophysin, Chromogranin, Neurofilaments, CD57, GFAP, Actin, Desmin, CD34, TTF-1 gave negative results. This immunoprofile was highly consistent with the poorly differentiated variant of synovial sarcoma, and the detection of the specific translocation t(X;18)(p11;q11) in the sample seemed to strongly support such a diagnosis. The additional peculiarity of this case is its intracranial location. Synovial sarcoma is known to occur in the head and neck region in about 5% of cases 1 and at least one intracranial case in a 19-years old woman has been reported in the literature 4 .
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 Bibliografía
1 Fisher C, de Bruijn DRH, Geurts Van Kessel. A “Synovial Sarcoma” in: Fletcher CDM, Unni KK, Mertens F Eds WHO Classification of Tumors “Pathology and Genetics of Tumours of Soft Tissue and Bone, pp. 200-204, IARC Press, Lyon, 2002

2 Pelmus M, Guillou L, Hostein I, Sierankowski G, Lussan C, Coindre JM Monophasic fibrous and poorly differentiated synovial sarcoma: immunohistochemical reassessment of 60 t(X;18)(SYT-SSX)-positive cases. Am J Surg Pathol. 2002 Nov;26(11):1434-40.

3 van de Rijn M, Barr FG, Xiong QB, Hedges M, Shipley J, Fisher C Poorly differentiated synovial sarcoma: an analysis of clinical, pathologic, and molecular genetic features. Am J Surg Pathol. 1999 Jan;23(1):106-12.

4 Kleinschmidt-DeMasters BK, Mierau GW, Sze CI, Breeze RE, Greffe B, Lillehei KO, Stephens JK Unusual dural and skull-based mesenchymal neoplasms: a report of four cases. Hum Pathol. 1998 Mar;29(3):240-5.
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