Case presentation

Katarina Odar, DMD, PhD

Institute of Pathology

Faculty of Medicine, University of Ljubljana

• 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

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

HISTOPATHOLOGY

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

DIAGNOSIS?

DIAGNOSIS

• MUCOEPIDERMOID CARCINOMA

• intermediate grade (grade II)

• lymph node status: 3/18

• ypT3N2Mx (stage group IIIB)

• no response to preoperative treatment

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

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

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

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?

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 %

• CRTC1-MAML2 gene translocation

(FISH, RT-PCR)

- diagnostically useful (70% MEC, mostly

low and intermediate grade)

- marker of favourable prognosis

• no data for EMEC

THANK YOU!