Head and Neck Tumor Conference: Lessons for the radiologist. Nothing to disclose. 62 y.o. man presents with a rough, patchy area along roof of mouth.

Transcription

1 Head and Neck Tumor Conference: Lessons for the radiologist Nothing to disclose David R. DeLone, MD Objectives To highlight clinical aspects of HN cancer cases that are not of immediate and obvious significance to the radiologist, but that s/he would probably still want to know about. 62 y.o. man presents with a rough, patchy area along roof of mouth.

2 Biopsy Malignant melanoma, clinically superficial spreading type with satellitosis. Path from gingival biopsy: Oral cavity, anterior maxillary gingiva, biopsy: Ulceration with focal atypical melanocytic hyperplasia. Surg path: DIAGNOSIS: Oral cavity, midline hard palate, wide local excision: Mucosal malignant melanoma, is identified over an ill-defined area of 2.4 x 1.5 cm. The depth of invasion is 1.1 mm. The surgical margins, after re-excision of the posterior margin, are negative for tumor. Lymph node, right neck sentinel, excision: A single right neck sentinel lymph node is negative for metastatic melanoma. Blue dye is not identified. Paraffin section immunostains for S-100, melan A, and tyrosinase are negative. Lymph node, right neck area II, excision: A single right neck area II lymph node is negative for metastatic melanoma. HN mucosal melanoma % of all melanoma, 6.3-8% of HN melanomas Presents two decades later than cutaneous Mean y.o. Pediatric rare, better outcomes Descending order of frequency Nasal- bulky, polypoid; turbinates, lateral wall > septum Oral cavity- flat, pigmented; hard palate, maxillary alveolus Sinuses- maxillary Pharynx- rare, possibly due to endodermal origin Melanocytes- neural crest- ectoderm Possible tobacco, formaldehyde risk for sinonasal MM Worse prognosis than cutaneous melanoma 5-yr DFS 0-20%, local and distant failure Gavriel, Melanoma Research, 2011 Oral melanosis Rare Controversial- Whether it s a precursor Whether it should be resected Whether it even needs close surveillance Can be secondary to imatinib Staging (AJCC 7) Note: There is no T1, T2, or Stage I or II. N1 is already Stage IVA. Wong, Dermatol Online J, 2011

3 Prognostic factors for DSS Clinical stage at presentation Tumor thickness > 5 mm Vascular invasion on histology Distant metastasis (Nodal metastasis lost significance on multivariate analysis.) Patel, Head & Neck, 2002 Nodal disease more common in oral MM Elective neck dissection reasonable Gavriel, Melanoma Research, 2011 Chemotherapy Cytotoxic doesn t improve survival GM-CSF BRAF mutations in < 10% of MM in HN KIT mutations in 15-30% of MM Receptor tyrosine kinase Imatinib Tumor regression 42% 1-year survival 50% Gavriel, Melanoma Research, 2011 Carvajal, JAMA, 2011 Guo, J Clin Oncol, 2011 Went on to have hot flashes and fatigue with GM-CSF, requiring dose reduction. Small fistula closed spontaneously 3 months from presentation, in situ recurrence Reexcised with repeat WLE Radiation Historic: melanoma thought to have greater capacity for DNA repair Hypofractionation Concern for late effects (Pfister JNCCN 2012) Possibly improved locoregional control Recommendation for gross residual disease 41 mos out recurrence Maxillectomy and prosthesis Positive margins grossly invisible Received 30 Gy, hypofractionated, for local control

4 2012 MFMER slide-19 Ipilimumabinduced hypophysitis Courtesy Dr. Geoff Fletcher Carpenter, AJNR, 2009 At 76 months growing lung nodules on PET/CT Too small for visible FDG accumulation FNA positive for lung metastasis Started ipilimumab, subsequent autoimmune colitis Disinhibits cytotoxic T-cell activation 2012 MFMER slide-20 Prognosis Nasal cavity 15-30% 5-yr survival Oral cavity 12.3% Sinuses 0-5% Survival continues to fall over years Potential for late recurrence Sinonasal melanoma Dauer, Oto HNS 2008 Anecdotally, can have protracted course, with recurrences separated by years 65 y.o. woman with long-standing inferior parotid mass Diagnostic considerations Well-circumscribed parotid tail mass Pleomorphic adenoma Warthin tumor Lymph node (lymphoma, met, reactive) Oncocytoma Low grade mucoepidermoid CA Acinic cell CA Hamilton, AJNR, 2003

5 Right superficial and inferior deep parotidectomy, SND levels I-III Right superficial and inferior deep parotidectomy, SND levels I-III DIAGNOSIS: A. Parotid, right, superficial, parotidectomy: Mammary analogue secretory carcinoma forming a 2.1 x 1.4 x 1.1 cm illdefined mass. Surgical resection margins are negative. Multiple (10) lymph nodes are negative for tumor. Photographed. B. Lymph nodes, right neck area I, excision: Multiple (4) lymph nodes are negative for tumor. C. Lymph nodes, right neck, dissection: Multiple (27) neck lymph nodes are negative for tumor including lymph nodes are the following levels: 1 level IB, 11 level IIA, 8 level IIB, and 7 level III. Mammary Analogue Secretory Carcinoma Secretory carcinoma of the breast juvenile breast cancer 3-15 y.o., occasionally elderly Recurrent balanced translocation t(12;15) (p13;q25) ETV6-NTRK3 ETV6 from Chr 12 and NTRK3 from Chr 15 Fusion gene encodes chimeric tyrosine kinase, which has transformational activity in epithelial and myoepithelial cells of murine mammary gland. Same histology occurs in the salivary glands Hormone receptor (AR/ER/PR) negative AR positivity is seen in Salivary duct carcinoma Carcinoma ex adenoma (SDC, other) others Ska lova, Am J Surg Pathol, 2010 Nasser, Am J Clin Pathol Mammary Analogue Secretory Carcinoma Lobulated histology can mimic acinic cell carcinoma No ETV6-NTRK3 in acinic cell ETV6-NTRK3 can also be seen in congenital fibrosarcoma, cellular mesoblastic nephromas, and AML Ska lova, Am J Surg Pathol, 2010 Joaquin Garcia, MD Mammary Analogue Secretory Carcinoma Cytoplasm and secretory material stain for mammaglobin, FISH for ETV6 rearrangement Mammary Analogue Secretory Carcinoma Painless mass most common presentation; hx 2 months to several years Soft palate, buccal mucosa, tongue base, lip 2 of 16 died of disease in Ska lova s series Joaquin Garcia, MD Chiosea, Histopathology, 2012

6 Oral Lichen Planus Who, without a DDS, can define this? Inflammatory disease Immunologic T-cell mediated process Women >40 Chronic (unlike skin) Concomitant disease Vaginal/vulvar 25% Cutaneous 15% Scalp, nails, esophagus, eyes Rarely regresses spontaneously Difficult to palliate Eisen, J Am Acad Derm, 2002 Oral Lichen Planus Reticular: incidental Erythematous and Erosive: Painful Oral squamous cell carcinoma 0.8% Buccal mucosa Gingiva Dorsal tongue Lateral and ventral tongue Lip, labial mucosa Possible HPV relationship, not tobacco/alcohol Eisen, J Am Acad Derm, 2002 Gorsky, OOOOE, MFMER slide y.o. man with 8 year history OLP, taking methotrexate, routine surveillance yields posterolateral tongue/floor of mouth lesion Underwent WLE FOM and tongue, ipsilateral MND levels I-IV. A. Floor of mouth, right, resection: Invasive grade 3 (of 4) squamous cell carcinoma is identified forming an ulcerated mass (1.5 x 0.8 x 0.3 cm). Maximum tumor thickness is 0.3 cm. Tumor invades the lamina propria. Angiolymphatic invasion is not identified. Surrounding mucosa shows mild to moderate dysplasia. Surgical margins are negative for carcinoma. Results of HPV and P16 studies will be reported in an addendum. In Situ Hybridization (ISH) studies performed on paraffin-embedded tissue sections for Human Papilloma Virus (HPV) DNA: HPV (family 6) ISH is Negative for types 6 and 11. HPV (family 16) ISH is Negative for types 16, 18, 31, 33, 35, 39, 45, 51, 52, 56, 58 and 66. B. Lymph nodes, right neck, dissection: Multiple (33) lymph nodes are negative for metastatic carcinoma including lymph nodes at the following levels: 2 level I, 3 level II, 15 level III, 13 level IV. The submandibular gland is unremarkable. No adenopathy 5 months from presentation: New painful nodule of right anterior tongue, SCC, right anterior partial glossectomy 8 months from presentation: multiple lesions, left lingual gingiva bx SCC, right parotid tail mass FNA SCC. Right parotidectomy, partial mandibulectomy, right SM node resection, left neck dissection. Adjuvant radiation, 60 Gy. 4 years from presentation: invasive poorly differentiated (grade 4 of 4) sarcomatoid squamous cell carcinoma along the right posterolateral tongue, wide local excision (no imaging). NED 3 months later

7 2012 MFMER slide y.o. man 1 month after Moh s surgery for a preauricular SCC, prior helical SCC x2. 3 x 3.5 cm Gr 2 SCC with perineural invasion, negative margins. Moh s site on PET, no evident adenopathy (glucose > 400). High Risk Skin Cancer (Squamous Cell) Location Lip Ear Scalp Forehead Temple Eyelid Nose Prior injury or chronic disease Size Depth Grade Perineural invasion Immunosuppression Alam, NEJM, MFMER slide-38 High Risk Skin Cancer (Squamous Cell) Size Ear Previous treatment Perineural invasion High Risk Skin Cancer Risk of lymph node metastasis Recurrent lesion Lymphovascular invasion* Inflammation Poor differentiation Perineural invasion Depth of invasion* Size of lesion * significant on multivariate analysis Alam, NEJM, 2001 Moore, Laryngoscope, 2005 High Risk Skin Cancer Risk of lymph node metastasis Recurrent lesion Lymphovascular invasion* Inflammation Poor differentiation Perineural invasion Depth of invasion* Size of lesion * significant on multivariate analysis 10 year survival < 20% with adenopathy < 10% with distant metastases Moore, Laryngoscope, 2005 Alam, NEJM, y.o. man 1 month after Moh s surgery for a preauricular SCC, prior helical SCC x2. 3 x 3.5 cm Gr 2 SCC with perineural invasion, negative margins. Moh s site on PET, no evident adenopathy (glucose > 400). Clinically NED at 19 months 2012 MFMER slide-42

8 Facial pain and facial weakness There is too often a delayed diagnosis as nononcologic causes are pondered... For months 2012 MFMER slide MFMER slide MFMER slide MFMER slide MFMER slide MFMER slide-48

9 2012 MFMER slide-49 Surgery Total parotidectomy Mastoidectomy, sacrificing the facial nerve Browlift, canthoplasty, temporalis transfer for oral suspension Right MRND levels I-V Parotid, right with facial nerve and neck levels I-V lymph nodes, total parotidectomy: High-grade adenocarcinoma ex-pleomorphic adenoma is identified forming an infiltrative, poorly circumscribed, 3.8 x 3.7 x 2.0 cm mass in the parotid gland. Tumor extends beyond the parotid gland and involves the facial nerve. Angiolymphatic and extensive perineural invasion are present. Invasive carcinoma is present at the anterior margin (inked). Multiple (42 of 42) right neck lymph nodes are involved by metastatic adenocarcinoma, some completely replaced by tumor, representing soft tissue nodules MFMER slide-50 Adjuvant therapy Palliative carboplatin and vinorelbine Altered due to persistent cytopenias PET 4 months post-op showed resolution of axillary nodes and improvement in operative bed PET 7 months post-op negative for FDG avid disease 6 weeks post-op 2012 MFMER slide MFMER slide y.o. woman with two years of progressive and persistent facial paralysis and facial pain 9 months post-op Started gemcitabine Further progression at 11 months, resumed carboplatin and vinorelbine 1 month 4 months 2012 MFMER slide MFMER slide-54

10 51 y.o. woman with two years of progressive and persistent facial paralysis and facial pain 12 months 17 months 19 months 2012 MFMER slide-55 Facial pain and facial weakness Trigeminal neuralgia and Bell s palsy are narrowly defined. Cancer until proven otherwise Trigeminal MR protocol Complete face protocol Coronal fiesta/ciss of cisternal trigeminal nerves and Meckel s caves Ax FLAIR and postgd T1 of brain (Ax postgd T1 IAC with fatsat) Facial weakness Siccoli, Lancet Neurol, MFMER slide-56