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Colecchia Who Non Germ Cell Tumors Update
Colecchia Who Non Germ Cell Tumors Update
MAURIZIO COLECCHIA
MILAN
Department of Pathology
IRCCS National Cancer Institute Milan
WHO 2004
Department of Pathology
IRCCS National Cancer Institute Milan
Department of Pathology
IRCCS National Cancer Institute Milan
Department of Pathology
• Rare tumors – 3-6% of testicular tumors in large series (AFIP, British Panel &Registry)
• Age of patients : 0-90 ; most cases between 5th and 6th decade ; 30 % in childhood
sclerosing variant
Sclerosing SCT Department of Pathology
From M Colecchia ed. “Pathology of IRCCS National Cancer Institute Milan
”
Testicular and Penile Neoplasms Springer , Basel 2016
Department of Pathology
Genetic profile
The most frequent chromosomal aberrations in one study
included gain of chromosome X (~40%) and much less
commonly losses of the entirety or part of chromosomes 2 and
19 {17333264}. Studies of Sertoli cell tumors of no specialized
type demonstrated CTNNB1 gene (which encodes β-catenin)
mutations in 70% {24061522} and also in a high proportion of
tumors with sclerosing morphology {25025367}.
(pp.216A)
12 % ( ? ) malignant ( 8 – 79 years )
a-inhibin
Lipid rich Sertoli cell tumor Department of Pathology
IRCCS National Cancer Institute Milan
Department of Pathology
IRCCS National Cancer Institute Milan
Sclerosing SCT
Tubular seminoma vs. SCT
Department of Pathology
IRCCS National Cancer Institute Milan
Department of Pathology
IRCCS National Cancer Institute Milan
ESCOP
Krakow
2012
Useful immunohistochemistry Department of Pathology
IRCCS National Cancer Institute Milan
SF-1 92 75 57 50 50 100
FOXL2 21 83 67 100 - -
ß-catenin 50 83 60 100 50 100
Inhibin 97 67 38 67 75 100
Melan A 94 67 75 50 50 100
Calretinin 97 56 75 44 75 100
WT-1 0 82 57 50 75 50
CD99 74 56 25 86 0 0
Synaptophyin 65 24 29 0 0 50
CK 22 78 17 43 67 -
S100 28 28 43 38 75 0
LCT = Leydig CT ; SCT = Sertoli CT; USCST = Unclassified CST; GCT = Granulosa CT
LCCST = Large cell calcifying SCT ; TTAG = Testicular tumor of adrenogenital syndrome
Department of Pathology
IRCCS National Cancer Institute Milan
Department of Pathology
IRCCS National Cancer Institute Milan
Carney syndrome
Peutz-Jeghers syndrome
Gynecomastia
Tuberous sclerosis
Androgen insensitivity syndrome
Large cell calcifying SCT Department of Pathology
IRCCS National Cancer Institute Milan
Courtesy
dr. MIKUZ
Department of Pathology
IRCCS National Cancer Institute Milan
Department of Pathology
IRCCS National Cancer Institute Milan
LCCST Department of Pathology
IRCCS National Cancer Institute Milan
Courtesy
dr. MIKUZ
Useful immunohistochemistry Department of Pathology
IRCCS National Cancer Institute Milan
SF-1 92 75 57 50 50 100
FOXL2 21 83 67 100 - -
ß-catenin 50 83 60 100 50 100
Inhibin 97 67 38 67 75 100
Melan A 94 67 75 50 50 100
Calretinin 97 56 75 44 75 100
WT-1 0 82 57 50 75 50
CD99 74 56 25 86 0 0
Synaptophyin 65 24 29 0 0 50
CK 22 78 17 43 67 -
S100 28 28 43 38 75 0
LCT = Leydig CT ; SCT = Sertoli CT; USCST = Unclassified CST; GCT = Granulosa CT
LCCST = Large cell calcifying SCT ; TTAG = Testicular tumor of adrenogenital syndrome
Department of Pathology
IRCCS National Cancer Institute Milan
Department of Pathology
IRCCS National Cancer Institute Milan
Most of the patients with TSCST had a favorable prognosis. Testis-sparing surgery may be feasible
and effective in case of small tumors. Few patients had metastatic spread, but only those with
nodal metastases may benefi t from an early retroperitoneal lymph node dissection. Risk factors
associate with disease behavior, but indications to prophylactic intervention remain
controversial.
Malignancy criteria Department of Pathology
IRCCS National Cancer Institute Milan
SF-1 92 75 57 50 50 100
FOXL2 21 83 67 100 - -
ß-catenin 50 83 60 100 50 100
Inhibin 97 67 38 67 75 100
Melan A 94 67 75 50 50 100
Calretinin 97 56 75 44 75 100
WT-1 0 82 57 50 75 50
CD99 74 56 25 86 0 0
Synaptophyin 65 24 29 0 0 50
CK 22 78 17 43 67 -
S100 28 28 43 38 75 0
LCT = Leydig CT ; SCT = Sertoli CT; USCST = Unclassified CST; GCT = Granulosa CT
LCCST = Large cell calcifying SCT ; TTAG = Testicular tumor of adrenogenital syndrome
Department of Pathology
Therapy
IRCCS National Cancer Institute Milan
Despite surgery, overall outcomes for Stage II appear to be poor due to the disease
Phenotype..
Most of the patients with TSCST had a favorable prognosis. Testis-sparing surgery may be feasible
and effective in case of small tumors. Few patients had metastatic spread, but only those with
nodal metastases may benefi t from an early retroperitoneal lymph node dissection. Risk factors
associate with disease behavior, but indications to prophylactic intervention remain
controversial.
Granulosa cell tumor of adult type Department of Pathology
IRCCS National Cancer Institute Milan
Granulosa cell tumor of adult type is a rare tumor with 50 cases reported to date.
The morphology does not differ from those arising in the ovary.
The tumor diameters range from 0.7 to 13 cm; the color is yellow.
Symptoms : Slow enlargement of the testis and, in 20%, gynecomastia are important
symptoms.
Serum inhibin and anti-mullerian hormone (AMH) can be elevated.
GCT –adult type case reports until 2014* Department of Pathology
IRCCS National Cancer Institute Milan
Schubert TE et al. Diagn Pathol. 2014;9:107. - * after our publication 5 more cases were reported
GCT –adult type case reports until 2014 Department of Pathology
IRCCS National Cancer Institute Milan
2 DOD (3mo and 11 y) 2AWD (6m and 6y) 13 NED (3mo and 17 y)
Granulosa cell tumor of adult type Department of Pathology
IRCCS National Cancer Institute Milan
Department of Pathology
IRCCS National Cancer Institute Milan
Schubert TE et al., Adult type granulosa cell tumor of the testis with a heterologous sarcomatous
Department of Pathology
IRCCS National Cancer Institute Milan
component:case report and review of the literature. Diagn Pathol. 2014 ;9:107
CK
CD99
Useful immunohistochemistry Department of Pathology
IRCCS National Cancer Institute Milan
SF-1 92 75 57 50 50 100
FOXL2 21 83 67 100 - -
ß-catenin 50 83 60 100 50 100
Inhibin 97 67 38 67 75 100
Melan A 94 67 75 50 50 100
Calretinin 97 56 75 44 75 100
WT-1 0 82 57 50 75 50
CD99 74 56 25 86 0 0
Synaptophyin 65 24 29 0 0 50
CK 22 78 17 43 67 -
S100 28 28 43 38 75 0
LCT = Leydig CT ; SCT = Sertoli CT; USCST = Unclassified CST; GCT = Granulosa CT
LCCST = Large cell calcifying SCT ; TTAG = Testicular tumor of adrenogenital syndrome
Granulosa cell tumor of juvenile type Department of Pathology
IRCCS National Cancer Institute Milan
The juvenile type is the most common testicular tumor in the first 6
months of life and accounts for 6% of all prepubertal testicular
Because of the age of the patients and, to a lesser degree, because of similarities,
the tumor is confused with the yolk sac tumor; this can be a tragic mistake, since
the juvenile granulosa tumor is absolutely benign and does not require any therapy
other than simple surgical enucleation.
Inhibin
Department of Pathology
Mixed forms and incompletely differentiated gonadal stroma tumors IRCCS National Cancer Institute Milan
Single cells can react with inhibin, but usually they are positive only for
smooth muscle actin and S-100 protein.
stromal tumor
Incompletely differentiated gonadal stroma tumor: Department of Pathology
S100 SMA
FOXL2 h-caldesmon
Gonadoblastoma Department of Pathology
From M Colecchia ed. “Pathology of IRCCS National Cancer Institute Milan
”
Testicular and Penile Neoplasms Springer , Basel 2016
Mixed germ cell stromal tumor : Gonadoblastoma Department of Pathology
IRCCS National Cancer Institute Milan
ESCOP
Krakow
2012
a-inhibin CAM 5.2 Department of Pathology
IRCCS National Cancer Institute Milan
Department of Pathology
IRCCS National Cancer Institute Milan
“…Gonadoblastoma is now
recognized as the only entity in
the mixed germ cell-sex cord
stromal category with the
unclassified form of germ cell-
sex cord stromal tumor
considered not sufficiently
established by the available
evidence.”
Department of Pathology
IRCCS National Cancer Institute Milan