Tumori mekih tkiva dojke
Tibor Tot
Falun, Švedska
Dani Medicinske Dijaspore, Beograd 2011
Epithelial
Myoepithelial
Mesenchymal Epithelial
M i x e d
M
i
x
e
d
Epithelial
Myoepithelial
Mesenchymal Epithelial
M i x e d
M
i
x
e
d
Osnovne činjenice
• Bilo koji tumor mekih tkiva se može pojaviti i u dojci;
• U poredjenju sa carcinomom dojke, tumori mekih tkiva u dojci su veoma retki;
• Veċa je verovatnoċa da jedan tumor sa izgledom sarcoma je u stvari metaplastični karcinom ili loše differentivani phylloides tumor.
Case courtesy of
Professor Vincenzo Eusebi,
Bologna, Italy Keratin
Metaplastic Carcinoma of the Breast
with Neuroectodermal Stromal Component
Tibor Tot, Juan Jose Badani De La Parra,
and Leif Bergkvist.
Pathology Research International 2011
CK MNF 116
Benigni tumori mekih tkiva
• Nespecifični za dojku
• ”Specifični” : Benign Stromal Spindle Cell
Tumors – BSSCTs
Tavassoli, Eusebi, Tumors of the Mammary Gland
AFIP Atlas of Tumor Pathology, Series 4, 2009
Nespecifični benigni tumori
• Lipoma
• Adenolipoma
• Angiomyolipoma (PECOMA)
• Angiolipoma
Lipoma
Capsule
Capsule
Capsule surrounding the circular / oval-shaped lesion
Capsule surrounding the circular / oval-shaped lesion
Male lipoma
Capsule surrounding the circular / oval-shaped lesion
Fibro-adeno-lipoma.
TDLUs
Fibrosis
Adipose tissue
Breast within the breast
Capsule
C
Capsule surrounding the circular / oval-shaped lesion
Fibro-adeno-lipoma or “breast within the breast”
Breast within the breast (fibro-adeno-lipoma): The fibroglandular tissue is radiopaque,
the adipose tissue is radiolucent.
• FAL
Breast within the breast
Capsule surrounding the circular / oval-shaped lesion
Fibro-adeno-lipoma
Lipoma
Nespecifični benigni tumori
• Angioma
• BLAP: Benign LymphAngiomatous
Papules of the skin after radiotherapy
Asymptomatic
Selected from screening
58 year
Right breast, upper lat
Fettily replaced breast
6x4mm circular lesion
Found also on ultrasound
Radiologic dg.:
Susp mucinous cancer
Haemangioma of the breast
Breast haemangioma:
Often asymptomatic
Incidental findings
Perilobular HA:
in 1,2% mastectomies
in 4,5% benign biopsies
in 11% autopsies
Rosen PP, Rindolfi RL.
Am J Clin Pathol 68:21-23, 1977
Breast haemangioma:
1. Symptomatic - Cavernous
- Capillary
- Venous
2. Perilobular
3. Atypical
WHO 2002
Mariscal A. et al.
Breast hemangioma
mimicking carcinoma.
Breast 2002 11:357-8.
Gopal SV et al.
Breast hemangioma
simulating an
inflammatory carcinoma
Breast J 2005 11:498-9
Galindo LM. et al.
Atypical hemangioma of
the breast: a diagnostic
pitfall in breast
fine-needle aspiration. Diagn Cytopathol 2001 24:215-8.
Honda SA, et al.
Hemangioma of the
breast with atypical
histological features.
further analysis of
histological subtypes
confirming their benign
character. Am J Surg Pathol 1992 16:553-60.
Nespecifični benigni tumori
• Angioma
• BLAP: benign lymphangiomatous papules
of the skin after radiotherapy
Case courtesy of
Professor Vincenzo Eusebi,
Bologna, Italy
Case courtesy of
Professor Vincenzo Eusebi,
Bologna, Italy
Nespecifični benigni tumori
• Nurofibroma, perineuroma, Schwannoma,
Granular cell tumor
• Case 1 diagnosed when the woman was
66 year old.
• No signs of recurrences
• As 80 she developed malignant lymphoma
and died of that disease.
Granular cell tumor of the breast
• 37 year old
• Palpable
• 10 mm
• Follow up 14 years, alive, no signs of
recurrence
S-100
S-100 NSE Cam5,2
Benign stromal spindle cell tumors
(BSSCTs)
• With predominant myofibroblastic
differentiation
• With predominant adipocytic component
• With prominent fibroblastic elements
• (Leiomyoma / variants)
BSSCT
SPINDLE CELLS
VIMENTIN +
CD 34+
ER;PR;AR
CLINICALLY BENIGN
ALL PUSHING BORDERS
BSSCTs with predominant
myofibroblastic differentiation
• Myofibroblastoma
• Solitary
• Any age
• Monosomy 16q&13q
Myofibroblastoma of the breast
BSSCTs with predominant
adipocytic component
• Spindle cell lipoma
Case courtesy of
Professor Vincenzo Eusebi,
Bologna, Italy
BSSCTs with
prominent fibroblastic elements
• Solitary fibrous tumor /
Hemangiopericytoma
Leiomyom dojke
Alpha smooth muscle actin
Maligni tumori mekih tkiva
• Nespecifični za dojku
• ”Specifični” : Malignant Stromal Spindle
Cell Tumors – MSSCTs
Tavassoli, Eusebi, Tumors of the Mammary Gland
AFIP Atlas of Tumor pathology, Series 4, 2009
Sarcomi dojke
• Veoma retki, < 1% od svih malignih
tumora
• Dr J Lamovec: 6/5382 malignih tumora u
Ljubljani za 1 godina
• Dalarna 2005-2011 oktobra, 1/1493
malignih slucajeva
Nespecifični maligni tumori
• Liposarcom
• Osteosarcom
• Rhabdomyosarcom
• Neurogeni maligni tumori
• (Angiosarcom)
Malignant Stromal Spindle Cell
Tumors – MSSCTs
• MSSCT with predominant fibroblastic
differentiation
• MSSCT with predominant myofibroblastic
differentiation
• (Leiomyosarcoma)
Vim SMA Ker CD34
MSSCT fibroblastic + - - +/-
MSSCT myofibroblastic + + - +
Monophasic
sarcomatoid carcinoma
+ - + -
Myoepithelial carcinoma
+ + -/+ -
Based on Tavassoli, Eusebi AFIP 4, page 291
The only difference resides in 25% of
axillary metastases in SC and virtually
never in sarcomas.
Treatment of MSSCTs
• Mastectomy (recurrences rare)
• Breast conserving surgery (recurrences in
up to 2/3 of cases)
• Survival ?
• Metastases to the lungs, liver, GI tract,
adrenals, brain, bones, pleura and
retroperitoneum.
MSSCT with predominant
fibroblastic differentiation
• Fibrosarcoma
• Malignant fibrous histiocytoma
• Vimentin only
• Diagnosis by exclusion
Case courtesy of
Professor Vincenzo Eusebi,
Bologna, Italy
MSSCT Fibroblastic
Low-grade High-grade
Growth pattern Herringbone Storiform
Mitosis 2/HPV 12/HPV
Necrosis -/+ ++
Nuclear atypia little variable
MSSCT Fibroblastic
Low-grade High-grade
Recurrences 63% 44%
Metastases none 25%
Deaths none 31%
Jones et al. Am.J.Surg.Pathol. 16: 667-674,1992
MSSCT with predominant myofibroblastic
differentiation
• Synonym: Malignant myofibroblastoma;
myofibrosarcoma.
• Vimentin +, SMA +, Keratin -
Case courtesy of
Professor Vincenzo Eusebi,
Bologna, Italy
Case courtesy of
Professor Vincenzo Eusebi,
Bologna, Italy
Leiomyosarcoma of the breast
• Positive for vimentin, actin& desmin
• Nearly 50% of cases around nipple
• Circumscribed margins
• Mitotic count no same impact as uterus
• Moderately aggressive neoplasms
• Complete excision the ONLY treatment
62 – year – old man with palpable lump
Erroneous FNAB diagnosis: Gynecomastia
Correct FNAB diagnosis: M y x o i d t u m o r
M y x o i d l i p o s a r c o m a of the male breast
Benign lipoma
Myxoid liposarcoma
Angiosarcoma
• Definition: Malignant tumors composed of
neoplastic elements with morphological
properties of normal endothelial cells.
• Synonym: hemangiosarcoma;
hemangioblastoma; lymphangiosarcoma
Angiosarcoma
• Primary (de novo) in breast parenchyma.
• Secondary in the skin and soft tissues of the arm following ipsilateral radical mastectomy and subsequent lymphedema (Stewart - Treves syndrome).
• Secondary in the skin and chest wall following radical mastectomy and local radiotherapy.
• Secondary in the skin or breast parenchyma or both following conservative treatment and radiotherapy.
Angiosarcoma - grade 1
Interanastomosing channels filled with red
blood cells.
Hyperchromatic nuclei
Dissection of interlobular and intralobular
stroma
Glands entrapped
Mitoses and necrosis absent
Donnel et al. Am J Surg Pathol 5:629-642,1981
Case courtesy of
Professor Vincenzo Eusebi,
Bologna, Italy
Angiosarcoma grade 3
Interastomosing vascular channels.
Solid areas (more than 50%)
Mitoses and necrosis present
Donnel et al. Am J Surg Pathol 5:629-642,1981
Angiosarcoma grade 2
Features of grade I in 75% of tumour.
Rest solid areas.
Endothelial tufting
Mitoses numerous
Donnel et al. Am J Surg Pathol 5:629-642,1981
TUFTING Case courtesy of
Professor Vincenzo Eusebi,
Bologna, Italy
Angiosarcoma - survival
5 - year 10 - year
Grade 1 91% 61%
Grade 2 68% 68%
Grade 3 31% 14%
Donnel et al. Am J Surg Pathol 5:629-642,1981
48 year-old woman with
palpable lump in her left breast
CD 34
CD 34
Pseudoangiomatous stromal hyperplasia
First report 1986
Vuitch MI, Rosen PP, Erlandson RA. Pseudoangiomatous
hyperplasia of the mammary stroma.
Human Pathol 1986;17:185-91
”prelymphatic” system linked to the main
lymph vessels in the breast
Hartveit F. Attenuated cells in breast stroma: the missing
lymphatic system of the breast.
Histopathology 1990;16:533-43
Tumor with myofibroblastic differentiation
Powell CM, Cranor LM, Rosen PP. Pseudoangiomatous
stromal hyperplasia (PASH). A mammary stromal tumor
with myofibroblastic differentiation.
Am J Surg Pathol 1995; 19:270-7.
Clinical manifestations:
1. Tumor forming (unilateral, firm, rubbery mass)
0,4 % of consecutive breast specimens Polger MR et al. Pseudoangoiomatous stromal hypeplasia: mammographic
and sonographic appearance. AJR 1996 166:349-52.
2. Incidental /microscopical
23 % of consecutive breast specimens Ibrahim RE et al. Pseudoangoiomatous hypeplasia of the mammary stroma;
some observations regarding its clinicoapathologic spectrum.
Cancer 1989 63 1154 -60
3. Massive bilateral (peau d’orange)
(1 case in Rosen’s Breast Pathology)
Clinical manifestations:
Massive (bilateral) (peau d’orange)
- 1 case in Rosen’s Breast Pathology
- 1 case: Mansouri D et al. Pseudoangiomatous stromal hyperplaia of mammary
stroma: a case with gigantomastia. Ann Pathol 2004; 24:179-82
+ 1
Tumor forming PASH: 1 male case
Seidman JD et al. Rapid growth of pseudoangiomatous hyperplasia
of mammary stroma in axillary gynecomastia in an immunosuppressed man.
Arch Pathol Lab Med 1993:117:736-8
PASH:
frequent incidental finding in gynecomastia
(23 – 47 %) Milanesi MF et al. Pseudoangiomatous hyperplasia of mammary stroma
associated with gynecomastia J Clin Pathol 1998:51 204-6
Badve S, Soane JP. Pseudoangiomatous stromal hyperplasia of male breast.
Histopathology 1995 26:463-6
Male, 25 y
Bilateral gynecomasti
following testosterone
intake
Radiologic appearance
A single case of round well-circumscribed tumor,
3.6 cm 8.2cm in 12 months
Taira N et al. Nodular pseudoangiomatous stromal hyperplasia of mammary
troma in a case showing rapid tumor growth. Breast Cancer 2005; 12:331-6
Most of the women are premenopausal,
If postmenopausal HRT
15 –55 (36) ys.
Puthi S et al.
Tamoxifen in the management of
pseudoangiomatous stromal hyperplasia.
Breast J 2001: 7:434-9
CD 34
CD 34
CD 34
CD 34
SMA
Normal interlobular stroma PASH
Normal interlobular stroma PASH
CD 34 CD 34
Normal interlobular stroma PASH
Tumors infiltrating PASH spaces
Damiani S, Eusebi V, Peterse JL. Malignant neoplasms
infiltrating pseudoangiomatous stromal hyperplasia of
the breast: an unrecognized pathway of tumour spread.
Histopathology 2002 41; 208-15.
- Collagenization of the interlobular stroma
- Empty anastomosing spaces
- Myofibroblasts resembling endothel,
CD 34 +
Actin +/-, Vim +,
CK-, FVIII-
progesterone +/-
Lui PC et al.
Fine-needle aspiration cytology of pseudo –
angiomatous stromal hyperplasia of the
breast. Diagn Cytopathol 2004 30:353-5.
Differential diagnosis:
1. Myofibroblastoma - Fascicular PASH
2. Low-grade angiosarcoma
PASH Myofibroblastoma (malignant)
Differential diagnosis:
1. Myofibroblastoma - Fascicular PASH
2. Low-grade angiosarcoma
CD31
PASH Angiosarcoma
Hvala !