Tumors122.docx

Tumors Case of large skin colored pinky tumor CD34 positive diagnosis: (DFSP) treatment a. Imatinib (800mg daily) b. Rituximab DFSP CD 34 positive : cKIT CDKN2A Mutation in ch ring BRAF v600 Biologic drug of melanoma which work on BRAF V600E Vemurafenib if not choose trametinib Ipilimumab Ni...

Tumors Case of large skin colored pinky tumor CD34 positive diagnosis: (DFSP) treatment a. Imatinib (800mg daily) b. Rituximab DFSP CD 34 positive : cKIT CDKN2A Mutation in ch ring BRAF v600 Biologic drug of melanoma which work on BRAF V600E Vemurafenib if not choose trametinib Ipilimumab Nivolumab Cobimetinib NB. Vemurafenib or Dabrafenib or Encorafenib target BRAF mutation Note that trametinib downstream of vemurafenib to inhibit MEK activation directly. And could affect BRAF V600E Trametinib or Cobimetinib or Binimetinib target MEK Ipilimumab target CTLA-4 Pembrolizumab or Nivolumab target PD-1 Imatinib and Nilotinib target C-KIT Biologic treatment of melanoma Stage 0, I , II , Respectable stage III - - Excision Unresectable stage III, stage IV, and recurrent melanoma In patients with metastasis less than 5 organs First line: programmed cell death–1 (PD-1) inhibitor pembrolizumab or nivolumab Second line use ipilimumab Third line: IL2 Fourth: chemotherapy. Dacarbazine Biologic drug of melanoma PD-1 negative a. Ipilimumab Melanoma in trunk associated with C KIT in acral and mucosa NARS in all types GRAS BRAF if in sun exposed Note: C KIT: mucosal, acral / BRAF: sun exposed / NRAS: all types)) Case melanoma, there is spread to LN treatment a. Ipilimumab Nivolumab pembrolizumab Doxorubicin Safety margin for melanoma with depth 3-5 mm 1 mm 3 mm 2 cm Note: safety margin of melanoma: In situ 0.5 cm 1 mm is 1 cm 3. 1.01-2 mm is 1-2 cm 4. More than 2 mm is 2 cm Picture dermoscopy of melanoma what treatment: Imatinib Rituximab Ipilimumab Melanoma 0.85 no ulceration what is next: a. Clarks Sentinel LN biopsy Moh’s surgery Ulcerated melanoma 0.85 next investigations: a. SLNB Case of malignant melanoma with Breslow depth of invasion 0.5mm safety margin of excision is 1cm Picture of Histopathology of skin tumor treatment surgical removal e safety margin 20 mm 50 mm 10 mm Note: safety margin of melanoma: In situ 0.5 cm 1 mm is 1 cm 3. 1.01-2 mm is 1-2 cm 4. More than 2 mm is 2 cm Case of malignant melanoma with Breslow depth of invasion 0.8mm safety margin of excision is 10 mm = 1cm 20 mm Dermoscopy of melanoma Case of melanoma, there is spread to LN bilateral, treatment Pembizumab Pembrolizumab Nivolumab Trametinib First line is nivolumab /pembrolizumab if bilateral affection Second line is Ipilimumab in unilateral LN affection Treatment of distal metastasis of melanoma: a. Nivolumab Dacarbazine Imatinib Picture of Lesion with rolled edges on cheek a. BCC SCC Melanoma Sebaceous carcinoma Picture of bcc and ask about action of mutant gene a. Inhibit smoothen if ask about gene function b. Stimulate smoothen if ask what gene act after mutation Note: PTCH 1 gene inhibit SMO normally but when mutated the inhibition is released so the SMO is stimulated Picture of skin nodule on face (merkle cell carcinoma) what is the treatment: Moh's surgery Surgical excision Radiotherapy Radiotherapy + surgical excision Picture of hair with nevus sebaceous most common tumor Trichoblastoma Picture of case of porokeratosis?? what is the most benign type Linear Punctuate (Bolognia) Disseminated superficial actinic keratosis Treatment of porokeratosis if disseminated: a. Retinoid PUVA MTX Patient with BCC and milia and trichoepithelioma a. Rombo if acral cyanosis Gorlin if frontal possing Bazex if hypohidrosis Female patient with scaly plaques on hands & feet. Nose & ear Ask about associated neoplasm. (Bazex syndrome) Lung Hepatic Nasopharynx Thyroid What is present in H/E of porokeratosis a. Parakeratosis Hyperkeratosis Orthokeratosis الجواب يعتمد على تكملة السؤال BCC: of multiple Case 27. May be rombo (BCCpositivermiculate hypotrichosis+ cyanosis + milia + atrophpoderma trichoepithellioma) AD Gorlin (AD + BCC+ medulloblastoma+ cyst in jaw + frontal possing) PATCH chromosome 9 gene affected Picture of Birt-hogg-Dube syndrome gene defect a. Folliculin (FLCN) on Ch17 AD Birt-hogg-Dube syndrome investigations Papillary Renal cell carcinoma المفروض (50% hybrid oncocytic as chromophobe / oncocystoma, then 34% chromophobe, then 9% clear cell, then 5% oncocystoma, then 2% papillary) GIT carcinoma Histological picture of fibrofolliculoma of birt hog dube ask about the disease name Female patient has skin colored papules her mother has history of leiomyoma pilar and uterine ,which type of cancer: Renal cell carcinoma Papillary carcinoma Clear cell carcinoma Spindle cell tumor immunohistochemical CD34 a. Positive BCC+ picture of dermoscope, biologic treatment a. Sonidegib Renal failure or carcinoma + keratotic papules or plaques in Elbows Calcium if calcinosis cutis Xray Angiography Female (red-brown or black??) nodule on back patient associated with Thyroid cancer: if macular amyloidosis Lung cancer: if it is metastasis or DFSP Adrenal cancer: if polycystic acne Leukaemia: if pyoderma gangernosum. Renal carcinoma: if pyogenic granuloma OR leiomyoma. Tumor scalp Lipoma Trichoblastoma Trichelemmeal cyst Histiocytosis + bone defect (Langerhans’s cell histiocytosis) a. Hand Schüller disease Letterer–Siwe disease Hand Schüller Christian disease Eosinophilic granuloma 0-2 years 2-6 years 7-12 years Visceral + bone D.I + exophthalmos + bone affection Primary bone affection Langerhans cell histiocytosis treatment Vinblastine + Prednisolone or Cytarabine If only skin treatment is by MTX, Thalidomide, Mercaptopurine (6-MP), Topical Nitrogen Mustard Note: BRAF- V600 inhibitor Vemurafenib, Dabrafenib. Picture of histopathology of SCC what is the site of lesion a. Lip Forehead Legs Note: body damaged by UV rays from the sun or tanning beds includes the head, neck, chest, upper back, ears, lips, arms, legs, hands Leiomyoma: Renal cell carcinoma Picture of surgeon removing 2 tumors from scalp with picture of histopathology, what is the lesion Atypical fibroxanthoma Tricheilemmal cyst Picture of brown lesion on the back for 6 weeks, what next a. Biopsy surgical removal Cryo antifungal Picture of old man face with ulcerating lesion on cheeks ( may be BCC cause border was rolled in) Keratoacanthoma BCC SCC Lesion brown in color in face with changes in color and shape and its size 10 mm what is next Incisional biopsy Excisional biopsy Shaving biopsy Picture of Histopathology: a. Syringoma b. Dermatofibroma Picture of surgeon removing 2 tumors from scalp with picture of histopathology) what is the lesion (trichilemmal cyst) Absence of granular cell layer Presence of granular cell layer Hyperpigmented macule with recently alternation in color reach 10 mm treatment Radiotherapy Surgery Biopsy Most common benign tumour from nevus sebaceous a. Trichoblastoma Most common malignant tumor from nevus sebaceous a. 20 % BCC Lesion on nose was found to be formed of basaloid cells and sebaceous gland what DNA repair gene missing MLH1/MSH2/MSH6/PMS2/ EPCAM. if Muir-Torre syndrome (Lynch syndrome ) Note: same question asks about DNA protein type a. 2 4 6 8 Old women with facial lesion blue cells with CD20 negative and CD7 positive CD99 negative: Merkle Ewing sarcoma Small lung SCC Actinic keratosis multiple best treatment Phototherapy Excision Curettage Cryotherapy Imiquimod Note cryotherapy is 1st line of treatment then 5FU then imiqumod. Patient on azathioprine & prednisolone with devolpement of iatrogenic Kaposi and no system affection what is next Stop the treatment Decrease dose of drugs Add chemotherapy for Kaposi Macule black brown grey invasion 0.8 and irregular margin treatment Shave excision Incisional biopsy Moh’s surgery Excisional biopsy Picture of scalp tumour seems keratoacanthoma treatment a. MTX Bleomycin Dacarbazine Picture of scalp lesion surgically removed Histopathology Increase stratum corneum Absent granular layer Presence of sebaceous gland Presence of apocrine gland Notes: Colloidal iron stains: Popular mucinosis, Scleroderma, Pretibial myxedema Mucin stain: Alcian blue, Toluidine blue, Colloidal iron Follicle centrE: (Bcl 6+ Bcl 2 -) Marginal zone: (Bcl 6- Bcl 2+) Diffuse large Leg type: (Bcl 2+ & 6 +) Biologic b cell lymphoma: Anti CD 20 (Rituximab) Urticaria pigmentosa aggravated by a. Dextromethorphan b. TMP Note: marker serum tryptase aggravated by anything cause mast cell degradation aspirin narcotics alcohol NSAI, Polymyxin B. If not in answers so choose increase prostaglandin metabolites A patient presents with recurrent crops of papules that ulcerate and then spontaneously heal. What immunohistochemical stain would be helpful in making the diagnosis?)Lymphomatoid papulosis( CD 4 CD 20 CD 30 CD 56 Lymphomatoid papulosis CD30 Translocation 21 Translocation 16 Translocation 16, 21 Note: Rearrangement of t(2,5) translocation t(8,9). Rosai Dorfman syndrome (neutrophilia, fever, anemia, elevated ESR and polyclonal hyper gamma globulineamia )and has (SC nodule) tumor. Ask about IMC. (CD14+, CD1a-, S100+/-, CD68+/CD207 (langerin) ) CD117 CD207 S100 CD1a Female with uterine leiomyoma and she has mass in lt arm Histopathology spindle cell history of mother having fibroid what type of tumor ? Reeds SyndromE Renal cell carcinoma Scenario or reed syndrome what type of renal cancer a. Papillary b. Chromophore 40 years female with generalized purpuric eruption all over body and sole ulcer Malignant melanoma Ulcerative lichen planus Same question treatment Mohs Cyclosporin Old men with mass in his face vimentin positive. S100 negative CD 68 positive,and spindle shaped cell What type of tumor: Atypical fibroxanthoma Primary Cutaneous Follicle Center Lymphoma BCL-6(+) and BCL-2(−) treatment i a. Radiotherapy Wide surgical excision Mohs surgery Rituximab Hedgehog pathway (PUMA /P21) Metastatic renal cancer positive for Ck 7 Ck 20 Pax 8 Rapid growth nodule in lower limb with bilateral inguinal LN treatment Imatinib Ipilimumab 2 Vismodigib Nivolumab 1 First line is Nivolumab / Pembrolizumab Second line is Ipilimumab Spiradenoma histopathology Picture Picture of lesion positive vimentin and negative all others pathology: spindle cells a. Atypical fibroxanthoma Scenario of lymphatoid papulosis and what is the commonest malignancy transformation CTCL Leukeamia CLL Hodgikin lymphoma Patient presents with recurrent crops of papules that ulcerate then spontaneously heal with hyperpigmentation CD30 positive. Treatment: if lymphomatoid papulosis Asymptomatic Steroids Phototherapy MTX if scars Male patient has plaque pigmented on his leg Kaposi and hhv8 positive need treatment a. Sirolimus Note: anti-HIV regimen: Surgery / Cryo / Interferon Histopathology (may be of syringoma) and need the diagnosis and the patient has same papules on face: Syringoma Microcytic adnexal carcinoma Photo of head of old patient has crusted lesion keratoacanthoma and need treatment injection of Bleomycin or MTX is better Rituximab Dacarbazine Description of highly progressive tumor in scalp Medulloblastoma Male patient 65 years old complain of sudden waxy brown papules on trunk.what cancer associated with this case? (seborrheic keratosis) Lung Liver Stomach Pancreas Trichoblastoma Description of lesion in vagina, investigation CT X ray Mammogram Thyroid scan Renal transplantation then after that has multiple skin ulcer and ask about cause a. CMV Patient with Sezary syndrome (syndrome ال وصف) on treatment should take contraception for how long 6 months 9 months 12 months 24 months Note: Generalized erythroderma, systemic therapies Low dose methotrexate: 3 to 6m Systemic retinoids Bexarotene: 1 m Brentuximab vedotin: 6m Interferon: 6m Romidepsin: 1m Vorinostat: 3-6m Alemtuzumab: 3 m but not confirmed PUVA therapy. Picture of small girl has small yellow nodule on scalp, what is another site for this lesion? Eye Liver Brain Lung Which chromosome associated with entity below (cylindroma) a. 16 17 15 20 X Single lesion of CBCL treatment Excision Intralesional Rituximab Radiotherapy Tumor on nose seems keratoacanthoma or sebaceous carcinoma, gene mutation Mismatch repair gene 1 Mismatch repair gene 2 Mismatch repair gene 3 Mismatch repair gene 4 Description of histopathology mimic PLEVA, treatment? Erythromycin Case Skin colored papule for long period , pneumothorax, associated with malignancy a. Kidney Case of mycosis fungoid patch stage 3 years duration, the best treatment? Nitrogen mustard topical Steroid Photo therapy Patient mycosis fungoides, patch stage for 3 years, what is the best treatment? Nitrogen mustard topical Steroid Phototherapy Histopathology of Warty Dyskeratoma, ask about common site a. Head and neck Histopathology of nevus sebaceous, ask about common site Head and face Tumour S100 negative P63 with pankeratin positive (SCC?)what is the Treatment ? Avelumab Cetuximab Mogamulizumab Nivolumab Case of Langerhans cell histiocytosis investigation needed x-ray on: Arm Spine Leg Cranium P53 mechanism a. Cell cycle G1 Cell cycle G2 Cell cycle S1 Mitosis Mechanism of action of P53 a. PUMA MDM2 (normally degrades P53) AKT (P13K AKT inhibit cell cycle arrest and apoptosis) CDKN2A (mutated gene in melanoma) Act by activation of P16 another CDK inhibitor, activation of P14ARF that inhibit MDM2. P53 Mutated in SCC-BCC mechanism of action Activation of P21(CDK inhibitor) lead to cell cycle arrest Activation of Puma that inhibit BCl2 apoptosis inhibitor there by lead to cell death Histopathology of spindle cells in a storiform pattern and what association Deletion of Ch 7 Deletion ch16 Translocation 17;21 Translocation 17;22 Dermoscope of bcc twice once with Bazex syndrome and the other need treatment Photo of keratoacanthoma ask about intralesional therapy Bleomycin b. MTX c. Cyclosporine Picture of skin colored solitary nodule under eye in child and Von Kossa stain positive, what association: Increase muscle Weakness as it is pilomatricoma Tonic colonic convulsion Muscle hypertrophy Mechanism of action of Vismodegib: Stimulate smoothed Inhibit smoothed Activate hedgehog pathway The protein product of the gene PTCH1 is which of the following? (Gorlin syndrome) (Board vital) A toll-like receptor A membrane-bound receptor Receptor kinase A G-protein-coupled receptor A ligand Explanation: The PTCH1 gene product, is a transmembrane protein. PTCH1 is mutated in Gorlin’s Syndrome, also known as Basal Cell Nevus Syndrome. It is characterized by autosomal dominant inheritance, mutation in PTCH1 gene coding the SHH sonic hedgehog membrane receptor protein. Key features include multiple basal cell carcinomas, medulloblastomas, palmar pits, jaw cysts, frontal bossing, bifid ribs, calcification of falx cerebri, agenesis of corpus collosum, hypertelorism, and ovarian fibromas / osteofibrosarcomas.