Germinal Tumors Classification in Oncology

General oncology. Classification of tumors Germinal tumors. Neuroectodermal tumors.

Classification of tumors 1. Epithelial tumors - surface epithelium/gland epithelium/special organs 1a. Neuroendocrine tumors 2. Mesenchimal tumors - Soft tissue/bones and cartilage/uncertain histog./undifferentiated 2a. Hematopoetic tumors 3. Neuroectodermal tumors 4. Germinal tumors

Biological behaviour of tumors Benign Precancerous Malignant

Germinal tumors

CNS Germinal tumors Tumors from totipotential stem cells. Mediastinum Can differentiate into extra-/somatic tissues = heterogenicity of tumors Retroperitoneum Gonads Migrates axially from yolk sac dorsally by axial line = performance Usually can be found at children and young adults

Germinal tumors Without differentiation With differentiation Seminomas Non-seminomas gonocytic Primitive embryonal Extra-embryonal Somatic Trophoblastic Seminomas Dysgerminomas Germinomas Teratomas Yolk sac tumor Embryonal carcinoma Choriocarcinoma Mature/ prepubertal teratoma Non-mature/ postpubertal teratoma Teratoma with somatic type of malignancy Mixed

Germinal tumors Precancerous lesion is non-invasive GCNIS (germ cell neoplasia in situ) Can be found only in testis (atypical gonocytes) Cryptorchismus/testicular feminization Type of tumor indicates biological behaviour+therapy+prognosis Seminomas CHTR sensitive Non-seminomas malignant types are more aggressive and resistant Isolated/mixed forms At mixed tumors the most aggressive component indicates therapy Volume of each component has an influence (checking by serum markers)

Germinal tumors. Seminomas. Seminoma = testis Dysgerminoma = extratesticular (ovaria) Germinoma = special title for CNS (epiphysis) Etiology Totipotential germ cells without differentiation Gonocyte character = primordial germ cell

Germinal tumors. Seminomas. Pathogenesis - Slow growth with invasion and mts - MTS lymphogenic retoperitoneal mediastinal cervical LN - MTS hematogenic later (lungs, liver, bones) Morphology - Macro grey-yellow solid lesion,+/- haemorrhage, necrosis - Micro solid made from polygonal gonocytes + lymphocytes + septas Clinics - Not painful tumor of gonads (at ovarium - stomachache) - Prognosis is great (due to CHRT sensitivity)

Germinal tumors. Embrional carcinoma. primitive carcinoma Etiology - Primitive embryonal differentiation into epithelial way (CK+) - Character of pluripotent stem cells Pathogenesis - Aggressive (isolated more, than mixed) MTS lymphogenic retroperitoneal mediastinal cervical LN MTS hematogenic lung, liver, bones Paraneoplasia -hCG+/- (pubertas praecox), LDH+/-, AFP+/-

Germinal tumors. Embrional carcinoma. Morphology Macro = similar to seminoma, but with bigger necrosis Micro = pleomorphic cells with vesicular nuclei with nucleoli with amorphic cytoplasmic + mitosis, apoptosis (different architectonic) Clinics - Similar to seminoma - Fast growth torsion of adnexa - Prognosis is bad without therapy

Germinal tumors. Yolk sac tumor. Tumor of endodermal sinus Etiology - Extraembrional differentioation with yolk sac way - Most often allantois/extraembryonic mezoderma/endodermal glands Pathogenesis - Aggressive MTS lymphogenic retroperitoneal mediastinal cervical LN MTS hematogenic lung, liver, bones Paraneoplasia AFP+, -hCG+/-, LDH+/-

Germinal tumors. Yolk sac tumor. Morphology - Macro = similar to seminoma - Micro = reticular growth with Schiller-Duval bodies ( primitive glomeruli ), hyalin globules with AFP deposites (PAS+) Clinics - Similar to seminoma - Prognosis is good with therapy

Germinal tumors. Chorioncarcinoma. Teratogenic, non-gestational/gestational Etiology - Extraembrional differentiation with trophoblastic way - Cytotrophoblast/syncytiotrophoblast/intermedial trophoblast Pathogenesis - One of the most aggressive tumors - MTS hematogenic: early (angioinvasion of trophoblast) + choriocarcinoma syndrome (visceral haemorrhage from mts); lung, liver, brain, GIT, spleen, adrenals - Paraeoplastic = -hCG (Male: gynecomastia, thyreotoxicosis, pregnancy test+. Female: pseudopubertas praecox, vaginal hemorrhage), LDH+/-, AFP-

Germinal tumors. Chorioncarcinoma. Morphology - Macro = characteristically with haemorrhages - Micro = tumor trophoblast without villi (max primitive without stroma), massive angioinvasion + always mixed Clinics - Aggressive with early dissemination - Prognosis is bad without therapy

Germinal tumors. Teratomas. Teratos monster Etiology - Somatic differentiation to derivates of 1-3 germinal lists endoderma/mezoderma/ ectoderma = mono-, bi-, tridermal teratomas Monodermal (monophasic) = character of (epi)dermoid cyst/struma ovarii Tridermal (triphasic) = almost malformations of fetus (fetiformal teratoma - homunculus) Pathogenesis - Benign and malignant behaviour with mts MTS lymphogenic regional LN MTS hematogenic lung, liver, bones No paraneoplasia

Germinal tumors. Teratomas. Morphology - Macro = cystic (mature)/solid (non-mature) - Cystic is the most common in ovarium, in testis is rare - Behaviour is influenced by maturity of teratoma - Mature/maturum/benign Chaotic skin, cartilage, bone, fat, muscle, teeth, eye, brain (can be a possibility of somatic type malignisation carcinoid ovari) - Non-mature/immaturum/malignant - Min one immature tissue (most common neuroectoderma)

Germinal tumors. Teratomas. Clinics Depends on localization (increased gonads/mediastinal syndrome/intracranial hypertension ) At male behaviour is influenced by age of patient Prepubertal (benign) even if immature Postpubertal (malignant) even if mature

Neuroectodermal tumors

Neuroectodermal tumors Come from neuroectoderma Neural tube CNS Neural crest PNS, melanocytes, medulla of adrenals, mesenchyme of head and neck

Neuroectodermal tumors 1. Tumors of CNS 2. Tumors of PNS Peripheral nerves (axons+Schwann cells+perineurium+fibroblasts) Neuronal ganglia (sensitive, sensorial, autonome; neuronal bodies) Paraganglia (close to ganglia sympatica+adrenal medulla/parasympatical nerves; chromaffin (symp - pheochromocytoma)/achromaffin (paras. - glomocytes)) 3. Melanocytic tumors

Neuroectodermal tumors 1. 2. Peripheral nerves - schwannoma, neurofibroma, MPNST - Perineuroma, neurothecoma, tumor from granular cells Tumors of CNS Tumors of PNS Neuronal ganglia - ganglioneuroma, ganglioneuroblastoma, neuroblastoma Paraganglia - paraganglioma, pheochromocytoma 3. Melanocytic tumors

Neuroectodermal tumors. Schwannoma Neurilemmoma, neurinoma Etiology From Schwann cells (neurilemmocytes) Sporadic/associated with NF 2 type Pathogenesis Benign without tendency to malignization Excentric growth from peripheral nerves

Neuroectodermal tumors. Schwannoma Morphology Macro most commonly head or spinal cord (but any nerve is possible) -vestibular neurinoma acustica (n.VIII), mono-/bilateral (NF 2t) - Spinal intradural extramedullar (often posterior sensitive roots) - Peripheral nerves of head, neck and extremities Micro localized (S100+) - Often with connective tissue capsule + lymphocytes and thick wall vessels - Antoni A palissaiding Verocai bodies - Antomi B myxoid stroma with microcystic changes - Can be degenerative atypia ancient schwannoma

Neuroectodermal tumors. Schwannoma Clinics Adults Vestibular tinnitus, hearing disorder/vestibular disorder, pressure to n.V, VII, cerebellum Spinal root signs up to compression of spinal cord Periferal - bulk

Neuroectodermal tumors. Neurofibroma 2 basical forms skin (diffuse)/plexiform Skin type (bulk) Etiology From Schwann cells+fibroblasts+perineurium Sporadic/ass. with NF 1 type Pathogenesis -benign with possibility to malignization Plexiform type (bag of worms)

Neuroectodermal tumors. Neurofibroma Morphology - Macro = localized lesion without capsule - Most common underskin/deeper nerves and plexi/visceral Micro: Schwann cells+fibroblasts+perinurium (S100+ 50%) -skin (diffuse): spindle cells with collagen/myxoid stroma -plexiform: diffuse interfascicle growth Clinics Children and adults Risk of nerve disorder at excise / cosmetic defect / malignization

Neuroectodermal tumors. MPNST Malignant tumor from peripheral nerve shneat, malignant shwannoma, neufibrosarcoma Etiology From Schwann cells with HG transformation Sporadic/malignization of neurofibroma Pathogenesis Malignant, local aggressive with MTS Hematogenic = lung

Neuroectodermal tumors. MPNST Morphology Macro widening of nerves up to infiltration of surrounding tissues Micro spindle cell HG tumor with S100+/- Mitosis/atypia/map-like necrosis Melanotic MMNST/epitelioid variant/ RMS dif (Triton tumor) Clinics Children and adults Prognosis is bad

Neuroectodermal tumors. Neuroblastoma Neuroblastoma ganglioneuoroblastoma ganglioneuroma Etiology From neuroblasts (precursors neurons of ganglia sympatika) Neural crest sympatogonia neuroblasts/feochromocytoblasts Pathogenesis Malignant with different localization by age Can be benign ganglioneuroma/spontaneus regression MTS lymphogenic = older children (LN+hem.bones) MTS hematogenic = newborns (liver, bones, skin blueberry muffin)

Neuroectodermal tumors. Neuroblastoma Morphology Macro = adrenal medulla Extraadrenal mediastinum and retroperitoneum Micro = from small dark round cells Immature neuroblasts with Homer-Wright rosettes Ganglioneuroblastoma = mature and immature component Ganglioneuroma = ganglion cells+Schwann cells = mature ganglion Clinics Children (30/year) Prognosis 15% of onco death Adults mostly have ganglioneuroma

Neuroectodermal tumors. Paragangliomas and pheochromocytoma Pheochromocytoma = paraganglioma of adrenal medulla Etiology From pheochromoblasts Neural crest sympatogonia neuroblasts pheochromoblasts Pathogenesis Malignant with mts potential +risk of hypertension crisis

Neuroectodermal tumors. Paragangliomas and pheochromocytoma Morphology Macro = adrenal medulla/paraganglia Sympatic paraganlias = Zuckerkan organ/parasympaticus glomus caroticum, jugulotympanicum, subclavium Micro = Zellballen main+sustencacular cells (S100+) IHC synaptophysin, chromogranin A Pheochromocytes Glomocytes Clinics Adults and young Hypertension