Teratocarcinosarcoma Rare Tumor Presentation.pptx

Teratocarcinosarcoma Rare Tumor Presentation.

1. RARE TUMOUR BOARD DR.RITAM JOARDER SENIOR RESIDENT 06-01-2025 Sinonasal Teratocarcinosarcoma

2. 49Yrs old lady R/o Bihar- Homemaker No comorbidities No addiction No FHOC Baseline complaints : Headache since last 3months B lockage of both nostril since last 2 months Decreased vision in Left Eye since last 2months

3. On examinations: PS: ECOG-1 B/L Level IA and Left Level III neck node palpable DNE- An ulceroproliferative growth on Left middle turbinate which extend superiorly and some bulge coming out from left Maxillary sinus ostium. An mass also present on right superior to middle meatus region which is non pulsatile. Hopkin examinations: NAD B/L Vocal cord -mobile

4. MRI PNS and Neck 21.11.2024 : -An ill-defined, soft tissue lesion is seen involving epicentered in the nasal cavity and the bilateral posterior ethmoid region extending superiorly upto the anterior and middle cranial fossa approx 3.1 x 3.3 x 5.8 cm. -The lesions indenting over medial wall of left Maxillary sinus, extending into maxillary ostium, medial wall of left orbit (in extraconal space). -Ant cranial fossa plaque like nodular dural thickening with suspicious intraparenchymal involvement in the left basifrontal region but reactive at planum sphenoidale. - B/L Level III necrotic LN largest 1.8x1.5cm -Retention of secretion in Left Maxillary, frontal and sphenoid sinus. -Altered marrow in the clivus without enhancement

5. NCCT Thorax 21 .1 1 .2024 : - Left Upper lobe 3mm nodule – TSTC - A hypodense subcapsular lesion in segment IV of the liver-measuring approx. 1.8x1.1cm

6. Histopathology report:

7. REVIEW OF LITERATURE

8. Epidemiology: Sinonasal teratocarcinosarcoma is a rare malignant tumour consisting all 3 germ layers of nasal cavity and para nasal sinuses. Commonly found in the nasal cavity, PNS, ovaries, or uterine cervix. Median age- 54.7 yrs Male : female – 7:1 to 8:1 Many SNTCS cases are diagnosed at an advanced stage, and the average 2- to 3- year survival rates range from 45% to 60%. Rao YF, et al. J Int Med Res. 2020

9. Clinical features: M.C.– nasal obstruction ( 75% ) , recurrent epistaxis ( 62.8% ), Headache If tumor invades the surrounding tissues –facial swelling, proptosis, optic atrophy, Most common sites of metastasis – lung , brain No known risk factor or tumor predisposition syndrome association. Nasal endoscopy CECT and MRI PNS shows soft tissue lesions in paranasal sinuses and nasopharynx with bony erosions and intracranial extension. CECT thorax + Abdomen to be done to rule out distant metastasis Evaluation:

10. Histopathology, IHC and Cytogenetics: Tumor comprises an admixture of epithelial, mesenchymal and neuroepithelial elements CK5/6 : cytoplasmic staining in neoplastic squamous epithelial cells Synaptophysin  and  chromogranin A : cytoplasmic staining in primitive neuroepithelial cells BRG1  stain: loss of nuclear expression in tumor cells Beta catenin : nuclear staining in neoplastic epithelial cells (aberrant pattern in small subset of TCS cases) ( Turk Neurosurg 2017;27:468 ,  J Neurol Surg B Skull Base 2017;78:346 ) Biallelic inactivation of  SMARCA4  (most common-65%) ( Cells 2023;13:81 ) Activating  CTNNB1  mutation (second most common-35%)

11. Treatment : Most common modality of treatment – surgery f/b chemoradiation therapy Transfacial approach is the mainstay surgical approach to all lesions of nose and sinonasal region Subcranial and transcranial approach for lesions with intracranial extension Anterior craniofacial resection , maxillectomy . Neoadjuvant chemotherapy for unresectable disease.

12. Systematic review of all available literature on SNTCS (from 1977–2018) N= 127 Patients. Male- 83%, median Age-50yrs (10-82) Majority (90%) u/w Surgery f/b adjuvant therapy 55% - Surgery f/b RT and 20% -Surgery f/b Adj.Chemotherapy Median time to recurrence -19.5months Recurrence rate was 38% and 2yrs survival was 55%. Multimodality treatment found to be superior to surgery alone. Chapurin N et al. Am Journal of Rhinology & Allergy 2021

13. -49articles representing 86cases. -Intracranial extension, cribriform plate and anterior cranial fossa involvement occurred in 20.9% patients. -Combined treatment with surgery, RT and chemotherapy resulted highest survival (88.8%) with no metastasis and lowest recurrence rate (22.2%). -With Surgery and RT (no chemotherapy) 26.1% cases recurred, 10.9% cases metastasized and 8.7% reported recurrence and metastasis. -In patients who were treated with either only CTRT (4cases) ,50% of them recurred. Mishra P et al. Am Journal of Rhinology 2014

14. Joshi A et al. JCRT 2015

15. Clinical Oncology 36 (2024) e137ee145 Retrospective audit to study role of NACT to downstage the disease, achieve complete resection, and impact on long-term survival outcomes Total no of patients – 27, Median age – 42 yrs ( 30-56 ) Predominantly male ( 85.2 % ) and female ( 11.1% ) 7 patients have palpable regional LN ( 25.9 % ) , 1 patient had lung metastasis , 15 patients ( 55.6 % ) –had intracranial extension 20/ 27 patients ( 74.1 % ) received etoposide and cisplatin as NACT Incidence of ≥ Grade 3 toxicity – 37% 18 patients underwent surgery , 5 patients received CTRT , 2 patients received definitive RT 2 yrs PFS was 53.2% and OS was 60.9%

16. MY PLAN - Targeted USG liver to characterize the liver lesion and guided biopsy from Liver lesion (if indicated) 3-4 cycles of Inj.Cisplatin (100mg/m2 D1) and Inj.Etoposide (100mg/m2 D1-3) q21days -> response assessment

17. Thank You