|
|
| Line 1: |
Line 1: |
| | __NOTOC__ |
| '''For patient information click [[{{PAGENAME}} (patient information)|here]]''' | | '''For patient information click [[{{PAGENAME}} (patient information)|here]]''' |
|
| |
|
| Line 13: |
Line 14: |
| DiseasesDB = | | | DiseasesDB = | |
| }} | | }} |
| {{SI}} | | {{Germ cell tumor}} |
| {{CMG}} | | {{CMG}} |
| __NOTOC__
| |
| {{Editor Help}}
| |
|
| |
| ==Overview==
| |
|
| |
| '''Germ cell tumor''' ('''GCT''') is a [[tumor]] ([[neoplasm]]) derived from [[germ cell]]s. (Germ cells themselves are not pathogenic; i.e., they are not the viral and bacterial "germs" that cause illness.) Germ cell tumors can occur both inside and outside of the gonads ([[ovary]] and [[testis]]).
| |
|
| |
| ==Etiology==
| |
|
| |
| Some investigators suggest that this distribution arises as a consequence of abnormal migration of germ cells during embryogenesis. Others hypothesize a widespread distribution of germ cells to multiple sites during normal embryogenesis, with these cells conveying genetic information or providing regulatory functions at somatic sites.
| |
|
| |
| Extragonadal germ cell tumors were thought initially to be isolated [[metastasis|metastases]] from an undetected primary tumor in a [[gonad]], but it is now known that many germ cell tumors are [[congenital]] and originate outside the gonads. The most notable of these is [[sacrococcygeal teratoma]], the single most common tumor diagnosed in babies at birth.
| |
|
| |
| ==Classification==
| |
|
| |
| Germ cell tumors are classified by their [[histology]],<ref name="pmid15761467">{{cite journal
| |
| | author = Ulbright TM
| |
| | title = Germ cell tumors of the gonads: a selective review emphasizing problems in differential diagnosis, newly appreciated, and controversial issues.
| |
| | journal = Mod. Pathol.
| |
| | volume = 18 Suppl 2
| |
| | issue =
| |
| | pages = S61-79
| |
| | year = 2005
| |
| | pmid = 15761467
| |
| | doi = 10.1038/modpathol.3800310
| |
| | issn =
| |
| }} {{PMID|15761467}} '''free full text'''</ref> regardless of location in the body.
| |
|
| |
| Germ cell tumors are broadly divided in two classes:<ref>{{cite web |url=http://www.emedicine.com/med/topic2246.htm |title=eMedicine - Germinoma, Central Nervous System : Article by Daniel D Mais, MD |accessdate=2007-11-03 |format= |work=}}</ref>
| |
|
| |
|
| * The '''germinomatous''' or '''seminomatous''' germ cell tumors (GGCT, SGCT) include only [[germinoma]] and its synonyms [[dysgerminoma]] and [[seminoma]].
| | ==[[Germ cell tumor overview|Overview]]== |
| * The '''nongerminomatous''' or '''nonseminomatous''' germ cell tumors (NGGCT, NSGCT) include all other germ cell tumors, pure and mixed.
| |
|
| |
|
| The two classes reflect an important clinical difference. Compared to germinomatous tumors, nongerminomatous tumors tend to grow faster, have an earlier mean age at time of diagnosis (~25 years versus ~35 years, in the case of [[testicular cancer]]s), and have a lower 5 year survival rate. The survival rate for germinomatous tumors is higher in part because these tumors are exquisitely sensitive to radiation, and they also respond well to chemotherapy. The prognosis for nongerminomatous has improved dramatically, however, due to the use of platinum-based chemotherapy regimens.<ref>Robbins, Basic Pathology; ISBN 0-7216-9274-5, 7th edition, pg 664.</ref>
| | ==[[Germ cell tumor historical perspective|Historical Perspective]]== |
|
| |
|
| '''Teratocarcinoma''' is an old name for a germ cell tumor that is a mixture of [[teratoma]] and [[embryonal carcinoma]]. In more modern usage, this kind of mixed germ cell tumor may be known as a teratoma with elements of embryonal carcinoma, or simply as an embryonal carcinoma.
| | ==[[Germ cell tumor pathophysiology|Pathophysiology]]== |
|
| |
|
| {| class="wikitable"
| | ==[[Germ cell tumor epidemiology and demographics|Epidemiology & Demographics]]== |
| |-
| |
| ! Tumor
| |
| | [[ICD-O]]
| |
| ! Peak Age (yr)
| |
| ! [[Benign]] or [[malignant]]
| |
| ! Histology
| |
| ! Tumor marker
| |
| |-
| |
| | [[Germinoma]] including [[dysgerminoma]] and [[seminoma]]
| |
| | 9060/3
| |
| | 40-50
| |
| | Malignant
| |
| | Sheets of uniform polygonal cells with cleared cytoplasm; lymphocytes in the stroma
| |
| | 10% have elevated [[Human chorionic gonadotropin|hCG]]<!-- are these all mixed tumors? -->
| |
| |-
| |
| | [[Embryonal carcinoma]]
| |
| | 9070/3
| |
| | 20-30
| |
| | Malignant
| |
| | Poorly differentiated, pleomorphic cells in cords, sheets, or papillary formation
| |
| | Pure tumors '''do not''' secrete hCG, [[alpha-fetoprotein|AFP]]
| |
| |-
| |
| | [[Endodermal sinus tumor]], also known as '''yolk sac tumor''' (EST, YST)
| |
| | 9071/3
| |
| | 3
| |
| | Malignant
| |
| | Poorly differentiated endothelium-like, cuboidal, or columnar cells
| |
| | 100% secrete AFP
| |
| |-
| |
| | [[Choriocarcinoma]]
| |
| | 9100/3
| |
| | 20-30
| |
| | Malignant
| |
| | Cytotrophoblast and syncytiotrophoblast without villus formation
| |
| | 100% secrete hCG
| |
| |-
| |
| | [[Teratoma]] including mature teratoma, [[dermoid cyst]], immature teratoma, teratoma with malignant transformation
| |
| | 9080/0-9080/3
| |
| | 0-3, 15-30
| |
| | Mature teratoma, dermoid cyst usually benign (but [[Watchful_waiting|follow-up]] required); others usually malignant
| |
| | Very variable, but "normal" tissues are common
| |
| | Pure tumors '''do not''' secrete hCG, AFP
| |
| |-
| |
| | [[Polyembryoma]]
| |
| | 9072/3
| |
| | 15-25
| |
| | ?
| |
| | ?
| |
| | ?
| |
| |-
| |
| | [[Gonadoblastoma]]
| |
| | 9073/1
| |
| | ?
| |
| | ?
| |
| | ?
| |
| | ?
| |
| |-
| |
| | Mixed
| |
| |
| |
| | 15-30
| |
| | Malignant
| |
| | Depends on elements present
| |
| | Depends on elements present
| |
| |}
| |
|
| |
|
| ==Location== | | ==[[Germ cell tumor risk factors|Risk Factors]]== |
|
| |
|
| Despite their name, germ cell tumors occur both within and outside the [[ovary]] and [[testis]].
| | ==[[Germ cell tumor screening|Screening]]== |
|
| |
|
| * [[head]]
| | ==[[Germ cell tumor causes|Causes of Germ cell tumor]]== |
| ** inside the [[cranium]] — [[pineal]] and suprasellar locations are most commonly reported
| |
| ** inside the [[mouth]] — a fairly common location for [[teratoma]]
| |
| * [[neck]]
| |
| * 1% to 5% in the [[mediastinum]] ([[mediastinal germ cell tumor]])
| |
| * [[pelvis]], particularly [[sacrococcygeal teratoma]]
| |
|
| |
|
| *[[ovary]] {{main|Ovarian cancer}}
| | ==[[Germ cell tumor differential diagnosis|Differentiating Germ cell tumor from other Diseases]]== |
| *[[testis]] {{main|Testicular cancer}}
| |
|
| |
|
| In '''females''', germ cell tumors account for 30% of ovarian tumors, but only 1 to 3% of [[ovarian cancer]]s in North America. In younger women germ cell tumors are more common, thus in patients under the age of 21, 60% of ovarian tumors are of the germ cell type, and up to one-third are [[malignant]]. In '''males''', germ cell tumors of the testis occur typically after puberty and are malignant ([[testicular cancer]]).
| | ==[[Germ cell tumor natural history|Natural History, Complications & Prognosis]]== |
| In [[neonates]], [[infants]], and children younger than 4 years, the majority of germ cell tumors are [[sacrococcygeal teratoma]]s.
| |
|
| |
|
| Persons with [[Klinefelter's syndrome]] have a 50 times greater risk of germ cell tumors (GSTs)<ref>Mediastinal germ cell tumor in a child with precocious puberty and Klinefelter syndrome. Gregory G. Bebb, Frederic W. Grannis, Jr, Isaac B. Paz, Marilyn L. Slovak, Robert Chilcote. Ann Thorac Surg 1998;66:547-548. [http://ats.ctsnetjournals.org/cgi/content/abstract/66/2/547 Abstract]</ref>. In these persons, GSTs usually contain nonseminomatous elements, present at an earlier age, and seldom are gonadal in location.
| | ==Diagnosis== |
| | [[Germ cell tumor history and symptoms|History & Symptoms]] | [[Germ cell tumor physical examination|Physical Examination]] | [[Germ cell tumor laboratory tests|Lab Tests]] | [[Germ cell tumor electrocardiogram|Electrocardiogram]] | [[Germ cell tumor chest x ray|Chest X Ray]] | [[Germ cell tumor CT|CT]] | [[Germ cell tumor MRI|MRI]] | [[Germ cell tumor echocardiography or ultrasound|Echocardiography or Ultrasound]] | [[Germ cell tumor other imaging findings|Other Imaging Findings]] | [[Germ cell tumor other diagnostic studies|Other Diagnostic Studies]] |
|
| |
|
| ==Prognosis== | | ==Treatment== |
| | | [[Germ cell tumor medical therapy|Medical Therapy]] | [[Germ cell tumor surgery|Surgery]] | [[Germ cell tumor primary prevention|Primary Prevention]] | [[Germ cell tumor secondary prevention|Secondary Prevention]] | [[Germ cell tumor cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Germ cell tumor future or investigational therapies|Future or Investigational Therapies]] |
| The 1997 International Germ Cell Consensus Classification<ref>International Germ Cell Consensus Classification: a prognostic factor-based staging system for metastatic germ cell cancers. International Germ Cell Cancer Collaborative Group. J Clin Oncol. 1997 Feb;15(2):594-603 [http://www.ncbi.nlm.nih.gov/sites/entrez?db=pubmed&cmd=search&term=9053482 PubMed abstract]</ref> is a tool for estimating the risk of relapse after treatment of malignant germ cell tumor.
| |
| | |
| A small study of ovarian tumors in girls<ref name="pmid17172084">{{cite journal
| |
| | author = Stankovic ZB, Djukic MK, Savic D, Lukac BJ, Djuricic S, Sedlecki K, Zdravkovic D | |
| | title = Pre-operative differentiation of pediatric ovarian tumors: morphological scoring system and tumor markers. | |
| | journal = J. Pediatr. Endocrinol. Metab.
| |
| | volume = 19
| |
| | issue = 10
| |
| | pages = 1231-8
| |
| | year = 2006
| |
| | pmid = 17172084
| |
| | doi =
| |
| | issn =
| |
| }}</ref> reports a correlation between [[cyst]]ic and benign tumors and, conversely, solid and malignant tumors. Because the cystic extent of a tumor can be estimated by ultrasound, MRI, or CT scan before surgery, this permits selection of the most appropriate surgical plan to minimize risk of spillage of a malignant tumor.
| |
| | |
| ==Research==
| |
| | |
| {{main|Cancer research}}
| |
| {{main|Clinical trial}}
| |
| | |
| Germ cell tumors of children are the subject of [[clinical research]] by the worldwide [[Children's Oncology Group]] (COG), in a number of studies coordinated by Dr. John Cullen, MD.<ref>[http://www.curesearch.org/our_research/research_stories/research_stories.aspx?id=1097 CureSearch.org press release re Germ Cell Cancer]</ref> | |
| | |
| Intracranial Germ Cell Tumors have been studied through the International CNS GCT Study Group. Under the direction of Jonathan Finlay, the program director, three international treatment studies have been initiated since 1990 with the goal to maintain a high rate of cure while minimizing the [[late effect]]s of treatment.
| |
|
| |
|
| ==References== | | ==References== |
| Line 173: |
Line 51: |
| * [http://www.cancer.gov/cancertopics/pdq/treatment/extragonadal-germ-cell/healthprofessional Extragonadal Germ Cell Tumors] | | * [http://www.cancer.gov/cancertopics/pdq/treatment/extragonadal-germ-cell/healthprofessional Extragonadal Germ Cell Tumors] |
| * [http://www.cancer.gov/cancertopics/pdq/treatment/ovarian-germ-cell/HealthProfessional Ovarian Germ Cell Tumors] | | * [http://www.cancer.gov/cancertopics/pdq/treatment/ovarian-germ-cell/HealthProfessional Ovarian Germ Cell Tumors] |
| * [http://www.health.am/cr/germ-cell-tumors/ Primary Germ Cell Tumors of the Thorax]
| |
| * [http://www.health.am/cr/malignant-germ-cell-tumors/ Malignant Mediastinal Germ Cell Tumors]
| |
| * [http://theoncologist.alphamedpress.org/cgi/content/full/5/4/312#R9 Intracranial Germ Cell Tumors The Oncologist, Vol. 5, No. 4, 312-320, August 2000]
| |
|
| |
|
| {{SIB}}
| |
| {{Soft tissue tumors and sarcomas}} | | {{Soft tissue tumors and sarcomas}} |
|
| |
|
| |
|
| [[Category:Oncology]] | | [[Category:Oncology]] |
| Line 187: |
Line 60: |
| [[Category:Medical research]] | | [[Category:Medical research]] |
| [[Category:Mature chapter]] | | [[Category:Mature chapter]] |
|
| |
| [[de:keimzelltumor]]
| |
| [[nl:kiemceltumor]]
| |
|
| |
|
| {{WikiDoc Help Menu}} | | {{WikiDoc Help Menu}} |
| {{WikiDoc Sources}} | | {{WikiDoc Sources}} |