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Presenter :Dr Prarabdh Singh
 59 Years male hailing from Basti
 P.w.c.o difficulty in swallowing food ,difficulty in breathing & cough since 6
months
 Voice change since last 6 months
 Weight loss 8 kg in last 3 month
 Habits:
 Bidi smoker 2 bundles /day since past 25 years had stopped since last 4 month
 Tobacco chewer
 Was initially planned for definitive chemoradiotherapy outside Took 3 cycles of
RT & then defaulted (No details available)
 Presented in our institution on 19.02.2023
 FOL findings:
 Lesion involving TVC & FVC ,AE folds
 B/l PFS free
 3.9x3.3X6.4 cm lesion epicentered in supraglottis
 Epiglottis, paraglottic space ,TVC & FVC & Pre-epiglottic
space: Involved
 Anterior & Posterior commissure: Involved
 Valleculae,subglottis,trachea :Not involved
 Thyroid cartilage erosion :Involved both laminae eroded
 Exolaryngeal spread: present
 Nodes: No significant enlarged cervical nodes
 M Status: No evidence of any distant mets
 Received 2# of NACT Paclitaxel+Cisplatin +OMCT
 Response assesement CT scan dated 15.03.2023 Partial response
 Underwent Total laryngectomy +B/l selective neck dissection (II-IV)
+TEP +Primary closure
Adjuvant radiotherapy to a dose
of 60Gy/30# from 13.06.2023 to
28.07.23 along with 6 cycles of
conc. Cisplatin (L.d :27.7.2023)
 Term Coined by Schmincke in Germany in 1921.
 Back in 1926 was first discovered by Marx in pyriform sinus.
 Calvet and Ferlito, in a large review of 2052 laryngeal cancer cases, documented a
rare LE.
 Initial names attributed were undifferentiated carcinoma of nasopharyngeal type,
undifferentiated carcinoma with lymphoid stroma .
 WHO has described as undifferentiated squamous cell carcinoma accompanied
with prominent reactive lymphoplasmacytic infiltrate.
1)Comprehensive literature review of 21 studies comprising 46 patients.
2)Time frame : 1968 -2018
3)Out of 36 studies 8 were excluded due to different histology & 7 were excluded due to
presence in other locations apart from larynx & hypopharynx
4)To study clinicopathological features ,diagnostic & treatment modalities were extracted
and analysed using SPSS.
 Median age of 64 years (range 40-82 years ).
 Most common subsites
a)Supraglottis in laryngeal cancer
b)Pyriform sinus in hypopharyngeal cancer
 Median follow-up was 36 months.
 Median survival time was 30 months.
1)5 Year Overall
survival
65%
2)5 Year Disease –free
survival
68%
3)Disease free survival a)Node Positive:65%
b)Node Negative:69%
P value:0.938
a)Distant mets +:25%
b)Distant mets - :81%
P value:0.0001
 Rare neoplasm0.2% occurrence rate {Bansal et al}
 Bimodal distribution  between 20-30 years & after 60 years
 Close association with EBV virus with high incidence of regional & distant
metastasis.
 Possible sites: oropharynx, larynx ,hypopharynx,trachea,salivary glands , oral
cavity& Sino nasal tract.
 LECs exist in two form
a)Pure LEC
b)Hybrid form( along with SCC )
 Viral association ?????
 EBV associations more commonly linked with LEC of salivary glands ,lungs
thymus & Stomach{Macmillan et al}.
 More association of LEC with HPV {Acuna et al}.
 Smoking and alcohol major factor(Andryk et al).
 Higher rate of p53 damage in LEC consistent with squamous cell carcinoma.
 Tumours further classified into -
a)p-16 positive/p-53 negative (Viral etiology)
b)p-16 negative /p-53 positive (non –viral/genetic etiology)
 Squamous or cylindrical epithelium with organised lymphoid tissue.
 Might mimic malignant melanoma & Non-Hodgkin’s lymphoma
 Cytokeratin expression more in LECs {Micheau et al}
 Historically defined as clumps of undifferentiated cells +dense inflammatory
infiltrates of plasma cells & lymphocytes.
 Histological picture similar to non-keratinizing nasopharyngeal
carcinoma.{Hammas et al}
 Most common symptoms are
dysphagia, hoarseness & cervical
mass.
 MC sub-site in pyriform sinus in
hypopharyngeal tumours.
 Within larynx , supraglottis was
affected more than other sub-sites
{Marioni et al}
 LECs are more commonly associated
with laryngoceles.
Courtesy slide : Faisal et al
 Surgery & radiotherapy forms cornerstone in management.
 Laryngeal LECs are radiosensitive Good control rates with radiation {Macmillan}
 Upfront radiotherapy leads to higher incidence recurrences {Stanley et al}
 75% risk of nodal metastasis & 25% of patients with disseminated disease. {Marioni
et al}
 Role of induction chemotherapy remains controversial  As per Kermani et al
neo-adjuvant chemotherapy reduce disease volume with response rate of 30% at
primary & 50% at regional nodes .
 Chemotherapy protocol in advanced case of LEC suggested by Bugada et al
”EXTREME” regimen comprising Cisplatin ,Cetuximab & fluorouracil.
 One of important differentials of LECs are large cell neuroendocrine tumours
(LCNEC) warrants Chemoradiotherapy {Greene & Lewis Et al}
 LECs are rare & aggressive neoplasms with low incidence & prevalence with high
Risk of occult metastasis & distant spread.
 Incidence of cervical spread (55%) & distant spread (18%).
 Association of viral etiology.
 Higher risk of cervical involvement warrants elective neck treatment.
 Survival improved with surgery & adjuvant treatment.
Lymphoepithelial carcinoma of larynx.pptx

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Lymphoepithelial carcinoma of larynx.pptx

  • 2.  59 Years male hailing from Basti  P.w.c.o difficulty in swallowing food ,difficulty in breathing & cough since 6 months  Voice change since last 6 months  Weight loss 8 kg in last 3 month
  • 3.  Habits:  Bidi smoker 2 bundles /day since past 25 years had stopped since last 4 month  Tobacco chewer  Was initially planned for definitive chemoradiotherapy outside Took 3 cycles of RT & then defaulted (No details available)
  • 4.  Presented in our institution on 19.02.2023  FOL findings:  Lesion involving TVC & FVC ,AE folds  B/l PFS free
  • 5.
  • 6.  3.9x3.3X6.4 cm lesion epicentered in supraglottis  Epiglottis, paraglottic space ,TVC & FVC & Pre-epiglottic space: Involved  Anterior & Posterior commissure: Involved  Valleculae,subglottis,trachea :Not involved  Thyroid cartilage erosion :Involved both laminae eroded  Exolaryngeal spread: present
  • 7.  Nodes: No significant enlarged cervical nodes  M Status: No evidence of any distant mets
  • 8.  Received 2# of NACT Paclitaxel+Cisplatin +OMCT  Response assesement CT scan dated 15.03.2023 Partial response  Underwent Total laryngectomy +B/l selective neck dissection (II-IV) +TEP +Primary closure
  • 9.
  • 10.
  • 11.
  • 12. Adjuvant radiotherapy to a dose of 60Gy/30# from 13.06.2023 to 28.07.23 along with 6 cycles of conc. Cisplatin (L.d :27.7.2023)
  • 13.  Term Coined by Schmincke in Germany in 1921.  Back in 1926 was first discovered by Marx in pyriform sinus.  Calvet and Ferlito, in a large review of 2052 laryngeal cancer cases, documented a rare LE.  Initial names attributed were undifferentiated carcinoma of nasopharyngeal type, undifferentiated carcinoma with lymphoid stroma .  WHO has described as undifferentiated squamous cell carcinoma accompanied with prominent reactive lymphoplasmacytic infiltrate.
  • 14. 1)Comprehensive literature review of 21 studies comprising 46 patients. 2)Time frame : 1968 -2018 3)Out of 36 studies 8 were excluded due to different histology & 7 were excluded due to presence in other locations apart from larynx & hypopharynx 4)To study clinicopathological features ,diagnostic & treatment modalities were extracted and analysed using SPSS.
  • 15.
  • 16.
  • 17.  Median age of 64 years (range 40-82 years ).  Most common subsites a)Supraglottis in laryngeal cancer b)Pyriform sinus in hypopharyngeal cancer  Median follow-up was 36 months.  Median survival time was 30 months.
  • 18. 1)5 Year Overall survival 65% 2)5 Year Disease –free survival 68% 3)Disease free survival a)Node Positive:65% b)Node Negative:69% P value:0.938 a)Distant mets +:25% b)Distant mets - :81% P value:0.0001
  • 19.  Rare neoplasm0.2% occurrence rate {Bansal et al}  Bimodal distribution  between 20-30 years & after 60 years  Close association with EBV virus with high incidence of regional & distant metastasis.  Possible sites: oropharynx, larynx ,hypopharynx,trachea,salivary glands , oral cavity& Sino nasal tract.  LECs exist in two form a)Pure LEC b)Hybrid form( along with SCC )
  • 20.  Viral association ?????  EBV associations more commonly linked with LEC of salivary glands ,lungs thymus & Stomach{Macmillan et al}.  More association of LEC with HPV {Acuna et al}.  Smoking and alcohol major factor(Andryk et al).  Higher rate of p53 damage in LEC consistent with squamous cell carcinoma.  Tumours further classified into - a)p-16 positive/p-53 negative (Viral etiology) b)p-16 negative /p-53 positive (non –viral/genetic etiology)
  • 21.  Squamous or cylindrical epithelium with organised lymphoid tissue.  Might mimic malignant melanoma & Non-Hodgkin’s lymphoma  Cytokeratin expression more in LECs {Micheau et al}  Historically defined as clumps of undifferentiated cells +dense inflammatory infiltrates of plasma cells & lymphocytes.  Histological picture similar to non-keratinizing nasopharyngeal carcinoma.{Hammas et al}
  • 22.  Most common symptoms are dysphagia, hoarseness & cervical mass.  MC sub-site in pyriform sinus in hypopharyngeal tumours.  Within larynx , supraglottis was affected more than other sub-sites {Marioni et al}  LECs are more commonly associated with laryngoceles. Courtesy slide : Faisal et al
  • 23.  Surgery & radiotherapy forms cornerstone in management.  Laryngeal LECs are radiosensitive Good control rates with radiation {Macmillan}  Upfront radiotherapy leads to higher incidence recurrences {Stanley et al}  75% risk of nodal metastasis & 25% of patients with disseminated disease. {Marioni et al}  Role of induction chemotherapy remains controversial  As per Kermani et al neo-adjuvant chemotherapy reduce disease volume with response rate of 30% at primary & 50% at regional nodes .  Chemotherapy protocol in advanced case of LEC suggested by Bugada et al ”EXTREME” regimen comprising Cisplatin ,Cetuximab & fluorouracil.  One of important differentials of LECs are large cell neuroendocrine tumours (LCNEC) warrants Chemoradiotherapy {Greene & Lewis Et al}
  • 24.  LECs are rare & aggressive neoplasms with low incidence & prevalence with high Risk of occult metastasis & distant spread.  Incidence of cervical spread (55%) & distant spread (18%).  Association of viral etiology.  Higher risk of cervical involvement warrants elective neck treatment.  Survival improved with surgery & adjuvant treatment.