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Scheda a cura di Marco Chilosi (GYM)
CLASSIFICAZIONE
WHO
Classificazione "istogenetica"
di
Muller-Hermelink
Tipo
AB Timoma Misto
Nel timoma AB coesistono aspetti di timoma di tipo A e di tipo
B.
Le aree "corticali" sono formate da aggregati di
cellule epiteliali a nucleo vescicoloso associate in varia misura a timociti
CD1a +, TdT+.
Le aree "midollari" sono costituite da cellule fusate
e scarsi timociti. L'espressione di citocheratine ad alto peso (CK 34bE12)
è elevata in tutto il tumore. La CK14 è espressa in modo variabile da zona a
zona.
EE

CK 34bE12

ricca
componente di timociti CD1a +

Nella
maggior parte dei timomi AB le cellule epiteliali esprimono in modo anomalo
antigene CD20. Questa anomala espressione è molto specifica ed è utilizzata
per la caratterizzazione di queste forme.
CD20

-
Am J Surg Pathol 1992 Oct;16(10):988-97
- Neoplastic epithelial cells in a subset of human
thymomas express the B cell-associated CD20 antigen.
Chilosi M, Castelli P, Martignoni G, Pizzolo G, Montresor E, Facchetti F,
Truini M, Mombello A, Lestani M, Scarpa A, et al.
Istituto di Anatomia Patologica, Universita di Verona, Italy.
A series of 36 human thymomas have been immunohistochemically analyzed using
a panel of antibodies recognizing B-cell markers including CD20. Most
thymomas exhibiting the cortical pattern, according to the criteria of
Marino and Muller-Hermelink, were characterized by areas of medullary
differentiation containing variable numbers of CD20+ B lymphocytes, thus
mimicking the medulla of normal thymus. On the other hand, B cells were
absent or rare in thymomas recognized as mixed using the same morphological
criteria. Surprisingly, we observed in most mixed thymomas variable numbers
of CD20+ spindle cells, characterized by long slender processes. Using
double-marker analysis we could demonstrate the epithelial nature of these
cells (expression of keratin and lack of lymphoid and B-cell-related markers).
The immunoreactivity of thymoma epithelial cells with L26, an antibody
widely used in the characterization of B-cell lymphomas, can represent a
drawback of practical relevance in the differential diagnosis of mediastinal
tumors.
Am J Surg Pathol 2001 Jan;25(1):111-20
Spindle
cell and mixed spindle/lymphocytic thymomas: an integrated clinicopathologic
and immunohistochemical study of 81 cases.
Pan CC, Chen WY, Chiang H.
Department of Pathology, National Yang-Ming University and Veterans General
Hospital-Taipei, Taiwan. ccpan@vghtpe.gov.tw
Forty-three cases of spindle cell thymoma (medullary, WHO type A) and 38
cases of mixed spindle/lymphocytic thymoma (WHO type AB) were studied for
their clinicopathologic and immunohistochemical characteristics. Three
histologic patterns of spindle cell thymoma were observed: short-spindled
(57%), long-spindled (31%), and micronodular (12%). The short-spindled
variant was composed of oval to short spindle cells commonly arranged in a
hemangiopericytic or microcystic pattern. The long-spindled variant chiefly
consisted of fibroblast-like epithelial cells mimicking fibrohistiocytic
neoplasms. The micronodular variant was characterized by small nests of
short spindle cells dispersed among a lymphoid stroma with frequent germinal
centers. All kinds of spindle cell could be admixed with lymphocyte-rich
"cortex"-like areas to constitute mixed spindle/lymphocytic
thymomas. Immunohistochemically, the epithelial cells in up to 70% of the
short-spindled and long-spindled variants of spindle cell thymoma and 90% of
mixed spindle/lymphocytic thymomas were positive for CD20, whereas the
epithelial cells in all micronodular spindle cell thymomas were negative.
All of the spindle cell thymomas and most of the mixed spindle/lymphocytic
thymomas in this study were found in stages I and II. Follow up of the
patients did not disclose relapse or mortality directly resulting from the
tumors. However, the prognosis of stage I and II spindle cell and mixed
spindle/lymphocytic thymomas did not significantly differ from those of
stage I and II thymomas of other types by a stage-matched survival analysis.
Our data showed that spindle cell and mixed spindle/lymphocytic thymomas are
distinctive in histologic pattern and immunohistochemical profile. When
interpreted within the context of staging, spindle cell and mixed spindle/lymphocytic
thymomas presenting in stages I and II most likely behave in an indolent
fashion.
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