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Case presentation
Katarina Odar, DMD, PhD
Institute of Pathology
Faculty of Medicine, University of Ljubljana
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• 70-year-old female
• tumor of the distal esophagus
• biopsy: squamous cell carcinoma, moderately differentiated
• radiotherapy, 2 cycles of chemotherapy – side effects
• response to th evaluated as poor
• operation: subtotal resection of the distal esophagus and cardia with regional lymph nodes
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MACROSCOPY
• stenosing tumor in the
distal esophagus
• 3 cm in diameter,
invading the entire
thickness of the
esophageal wall
• margins free
• 18 regional lymph
nodes
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HISTOPATHOLOGY
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FOLLOW-UP
• death 3 weeks after surgery
• autopsy:
- diffuse alveolar damage
- bronchopneumonia
- acute necrotising pancreatitis
- acute intestinal infarction
• no residual carcinoma, no lymph node anddistant metastasis
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DIAGNOSIS?
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DIAGNOSIS
• MUCOEPIDERMOID CARCINOMA
• intermediate grade (grade II)
• lymph node status: 3/18
• ypT3N2Mx (stage group IIIB)
• no response to preoperative treatment
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MEC OF THE ESOPHAGUS (EMEC)
• MEC – common in head and neck region
• EMEC rare – 130 reported cases
• incidence 0.5 % - 2.9 %
• mean age 60 years (40-91), M:F 3:1
• upper, middle and lower esophagus
• clinical and macroscopic presentations do not differ
from other types of esophageal carcinoma
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EMEC
- definition
• nomenclature, definition and histogenesis not
well defined
• „Adenocarcinoma“ - „rare carcinoma with an
intimate mixture of squamous cells, mucus-
secreting cells and cells of an intermediate
type“ (WHO Classification of Tumours of the Digestive system, 2010)
• histogenesis? esophageal glands vs surface
squamous epithelium
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EMEC
- differential diagnosis• tumors with squamous and adeno- carcinomatous
component - main dd: MEC vs adenosquamous carcinoma
• favoring ASC:
- clear separation between adeno and SCC component
- connection with the surface
- extensive keratinisation
- more atipia
• SCC with focal glandular elements, AC with squamous metaplasia
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EMEC
- treatment and prognosis
• treatment of choice: surgery
• no beneficial effect of radio-/chemotherapy
• very few data on prognostic factors in EMEC
• tumor grade important prognostic factor in MEC
• EMEC: no standardised grading system
• head and neck MEC: grading system well defined
• grading of EMEC by the criteria established for MEC of the head and neck?
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MEC head and neck gradingLow grade (I) Intermediate grade (II) High grade (III)
- many cysts
- many mucous cells
- rare mitoses
- bland cytology
- locally invasive well
circumscribed
- no perineural, vascular
- 5 year >90%
- some cysts
- some mucous cells
- few or some mitoses
- slight to moderate atypia
- more infiltrative
- perineural, vascular
more often
- 5 year approx 70 %
- few cysts
- few mucous cells
- many mitoses
- significant atypia
- infiltrative
- perineural, vascular
- necroses
- 5 year approx 20 %
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• CRTC1-MAML2 gene translocation
(FISH, RT-PCR)
- diagnostically useful (70% MEC, mostly
low and intermediate grade)
- marker of favourable prognosis
• no data for EMEC
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THANK YOU!