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Germ cell tumor
 
 
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A germ cell tumor (GCT) is a neoplasm derived from germ cell
Germ cell

Germ cells are progenitors of the gametes. These singled-out cells move through the gut to the developing gonads and undergo mitotic Cell proliferation followed by meiosis and Cellular differentiation into either eggs or sperm ....
s. Germ cells normally occur inside the gonads (ovary
Ovary

The ovary is an ovum-producing reproductive organ, often found in pairs as part of the vertebrate female reproductive system. Ovaries in females are homology to testicle in males, in that they are both gonads and endocrine glands....
 and testis). Germ cell tumors that originate outside the gonads may be birth defects resulting from errors during development
Development

Development may refer to:...
 of the embryo
Embryo

An embryo is a multicellular organism ploidy eukaryote in its earliest stage of development, from the time of first cell division until birth, Egg , or germination....
.

Etiology
Some investigators suggest that this distribution arises as a consequence of abnormal migration of germ cells during embryogenesis.






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A germ cell tumor (GCT) is a neoplasm derived from germ cell
Germ cell

Germ cells are progenitors of the gametes. These singled-out cells move through the gut to the developing gonads and undergo mitotic Cell proliferation followed by meiosis and Cellular differentiation into either eggs or sperm ....
s. Germ cells normally occur inside the gonads (ovary
Ovary

The ovary is an ovum-producing reproductive organ, often found in pairs as part of the vertebrate female reproductive system. Ovaries in females are homology to testicle in males, in that they are both gonads and endocrine glands....
 and testis). Germ cell tumors that originate outside the gonads may be birth defects resulting from errors during development
Development

Development may refer to:...
 of the embryo
Embryo

An embryo is a multicellular organism ploidy eukaryote in its earliest stage of development, from the time of first cell division until birth, Egg , or germination....
.

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 metastases
Metastasis

Metastasis , or Metastatic disease, sometimes abbreviated mets, is the spread of a disease from one Organ or part to another non-adjacent organ or part....
 from an undetected primary tumor in a gonad
Gonad

The gonad is the organ that makes gametes. The gonads in males are the testes and the gonads in females are the ovaries. The product, gametes, are haploid germ cells....
, 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
Sacrococcygeal teratoma

Sacrococcygeal teratoma is a teratoma located at the base of the coccyx . It is thought to be a derivative of the primitive streak....
, the single most common tumor diagnosed in babies at birth.

Classification


Germ cell tumors are classified by their histology
Histology

Histology is the study of the anatomy of cell and tissue of plants and animals. It is performed by examining a thin slice of tissue under a light microscope or electron microscope....
, regardless of location in the body.

Tumor ICD-O Peak Age (yr) Benign
Benign

A benign tumor is a tumor that lacks all three of the malignant properties of a cancer. Thus, by definition, a benign tumor does not grow in an unlimited, aggressive manner, does not invade surrounding tissue , and does not metastasize....
 or malignant
Malignant

Malignant is a medical term used to describe a severe and progressively worsening disease. The term is most familiar as a description of cancer....
 Histology Tumor marker
Germinoma
Germinoma

A germinoma is a type of germ cell tumor which is not differentiated upon examination. It may be benign or malignant....
 including dysgerminoma and seminoma		 9060/3 40-50 Malignant Sheets of uniform polygonal cells with cleared cytoplasm; lymphocytes in the stroma 10% have elevated hCG
Human chorionic gonadotropin

Human chorionic gonadotropin is a glycoprotein hormone produced in pregnancy that is made by the the developing embryo soon after Conception and later by the syncytiotrophoblast ....
Embryonal carcinoma
Embryonal carcinoma

The Embryonal Carcinoma is one of the several types of testicular germ cell tumors, which also include the seminoma , teratoma, Endodermal sinus tumor and choriocarcinoma....
 9070/3 20-30 Malignant Poorly differentiated, pleomorphic cells in cords, sheets, or papillary formation Pure tumors do not secrete hCG, AFP
Alpha-fetoprotein

Alpha-fetoprotein is a protein which in humans is encoded by the AFP gene.This gene encodes alpha-fetoprotein, a major plasma protein produced by the yolk sac and the liver during fetal life....
Endodermal sinus tumor
Endodermal sinus tumor

Endodermal sinus tumor , also known as yolk sac tumor , is a member of the germ cell tumor group of cancers. It is the most common testicular tumor in children under 3, and is also known as infantile embryonal carcinoma....
, also known as yolk sac tumor (EST, YST)		 9071/3 3 Malignant Poorly differentiated endothelium-like, cuboidal, or columnar cells 100% secrete AFP
Choriocarcinoma
Choriocarcinoma

Choriocarcinoma is a malignant, trophoblastic and aggressive cancer, usually of the placenta. It is characterized by early hematogenous spread to the lungs....
 9100/3 20-30 Malignant Cytotrophoblast and syncytiotrophoblast without villus formation 100% secrete hCG
Teratoma
Teratoma

A teratoma is a kind of tumor . Definitive diagnosis of a teratoma is based on its histology: a teratoma is a tumor with biological tissue or organ components resembling normal derivatives of all three germ layers....
 including mature teratoma, dermoid cyst
Dermoid cyst

A dermoid cyst is a cystic teratoma that contains developmentally mature skin complete with hair follicles and sweat glands, sometimes luxuriant clumps of long hair, and often pockets of sebum, blood, fat, bone, Nail , teeth, eyes, cartilage, and thyroid tissue....
, immature teratoma, teratoma with malignant transformation		 9080/0-9080/3 0-3, 15-30 Mature teratoma, dermoid cyst usually benign (but follow-up
Watchful waiting

Watchful waiting is an approach to a medicine problem in which time is allowed to pass before medical intervention or therapy is used. During this time, repeated medical test may be performed....
 required); others usually malignant		 Very variable, but "normal" tissues are common Pure tumors do not secrete hCG, AFP
Polyembryoma
Polyembryoma

Polyembryoma is a rare, very aggressive form of germ cell tumor usually found in the ovaries.It has been found in association with Klinefelter syndrome....
 9072/3 15-25 ? ? ?
Gonadoblastoma
Gonadoblastoma

A gonadoblastoma is a complex neoplasm composed of a mixture of gonadal elements, such as large primordial germ cells, immature Sertoli cells or granulosa cells of the sex cord, and gonadal stromal cells....
 9073/1 ? ? ? ?
Mixed  15-30 Malignant Depends on elements present Depends on elements present


Germ cell tumors are broadly divided in two classes:

  • The germinomatous or seminomatous germ cell tumors (GGCT, SGCT) include only germinoma
    Germinoma

    A germinoma is a type of germ cell tumor which is not differentiated upon examination. It may be benign or malignant....
     and its synonyms dysgerminoma and seminoma
    Seminoma

    Seminoma is one type of testicular cancer that is believed to originate from the Germinal epithelium of the seminiferous tubules....
    .
  • 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
Testicular cancer

Testicular cancer is cancer that develops in the testicles, a part of the male reproductive system.In the United States, between 7,500 and 8,000 diagnoses of testicular cancer are made each year....
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.

Mixed

Mixed germ cell tumors occur in many forms. Among these, a common form is teratoma with endodermal sinus tumor.

Teratocarcinoma refers to a germ cell tumor that is a mixture of teratoma
Teratoma

A teratoma is a kind of tumor . Definitive diagnosis of a teratoma is based on its histology: a teratoma is a tumor with biological tissue or organ components resembling normal derivatives of all three germ layers....
 with embryonal carcinoma
Embryonal carcinoma

The Embryonal Carcinoma is one of the several types of testicular germ cell tumors, which also include the seminoma , teratoma, Endodermal sinus tumor and choriocarcinoma....
, or with choriocarcinoma
Choriocarcinoma

Choriocarcinoma is a malignant, trophoblastic and aggressive cancer, usually of the placenta. It is characterized by early hematogenous spread to the lungs....
, or with both. This kind of mixed germ cell tumor may be known simply as a teratoma with elements of embryonal carcinoma or choriocarcinoma, or simply by ignoring the teratoma component and referring only to its malignant component: embryonal carcinoma and/or choriocarcinoma.

Location


Despite their name, germ cell tumors occur both within and outside the ovary
Ovary

The ovary is an ovum-producing reproductive organ, often found in pairs as part of the vertebrate female reproductive system. Ovaries in females are homology to testicle in males, in that they are both gonads and endocrine glands....
 and testis.

  • head
    Head

    In anatomy, the head of an animal is the rostral part that usually comprises the brain, eyes, ears, nose, and mouth . Some very simple animals may not have a head, but many bilateria do....
    • inside the cranium — pineal and suprasellar locations are most commonly reported
    • inside the mouth
      Mouth

      The mouth, buccal cavity, or oral cavity is the first portion of the alimentary canal that receives food and begins digestion by mechanically breaking up the solid food particles into smaller pieces and mixing them with saliva....
       — a fairly common location for teratoma
      Teratoma

      A teratoma is a kind of tumor . Definitive diagnosis of a teratoma is based on its histology: a teratoma is a tumor with biological tissue or organ components resembling normal derivatives of all three germ layers....
  • neck
    Neck

    The neck is the part of the body on many limbed vertebrates that distinguishes the head from the torso or trunk. The scientific term signifying "of the neck" is nuchal....
  • 1% to 5% in the mediastinum
    Mediastinum

    The mediastinum is a non-delineated group of structures in the thorax , surrounded by loose connective tissue. It is the central compartment of the thoracic cavity....
     (mediastinal germ cell tumor
    Mediastinal germ cell tumor

    Malignant mediastinal germ cell tumors of various histologies were first described as a clinical entity approximately 50 years ago. mediastinum and other extragonadal germ cell tumors were initially thought to represent isolated metastasis from an inapparent gonads primary site....
    )
  • pelvis
    Pelvis

    The pelvis or pelvic girdle is the irregular bone structure located at the base of the spine . In the adult human, it is formed by the sacrum and the coccyx, the caudal part of the axial skeleton, and a pair of hip bones, part of the appendicular skeleton or human leg....
    , particularly sacrococcygeal teratoma
    Sacrococcygeal teratoma

    Sacrococcygeal teratoma is a teratoma located at the base of the coccyx . It is thought to be a derivative of the primitive streak....


  • ovary
    Ovary

    The ovary is an ovum-producing reproductive organ, often found in pairs as part of the vertebrate female reproductive system. Ovaries in females are homology to testicle in males, in that they are both gonads and endocrine glands....
     
  • testis


In females, germ cell tumors account for 30% of ovarian tumors, but only 1 to 3% of ovarian cancer
Ovarian cancer

Ovarian cancer is a malignant tumor arising from an ovary. Although ovarian cancer is known to occur in many species, the majority of the medical literature and the focus of this article is on ovarian cancer in humans....
s in North America
North America

North America is the northern continent of the Americas, situated in the Earth's northern hemisphere and almost totally in the western hemisphere....
. 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
Malignant

Malignant is a medical term used to describe a severe and progressively worsening disease. The term is most familiar as a description of cancer....
. In males, germ cell tumors of the testis occur typically after puberty and are malignant (testicular cancer
Testicular cancer

Testicular cancer is cancer that develops in the testicles, a part of the male reproductive system.In the United States, between 7,500 and 8,000 diagnoses of testicular cancer are made each year....
). In neonates, infants, and children younger than 4 years, the majority of germ cell tumors are sacrococcygeal teratoma
Sacrococcygeal teratoma

Sacrococcygeal teratoma is a teratoma located at the base of the coccyx . It is thought to be a derivative of the primitive streak....
s.

Males with Klinefelter's syndrome
Klinefelter's syndrome

Klinefelter's syndrome, 47,XXY or XXY syndrome is a condition in which males have an extra X sex chromosome.While females have an XX chromosomal makeup, and males an XY, Affected individuals have at least two X chromosomes and at least one Y chromosome....
 have a 50 times greater risk of germ cell tumors (GSTs). In these persons, GSTs usually contain nonseminomatous elements, present at an earlier age, and seldom are gonadal in location.

Prognosis


The 1997 International Germ Cell Consensus Classification is a tool for estimating the risk of relapse after treatment of malignant germ cell tumor.

A small study of ovarian tumors in girls reports a correlation between cyst
Cyst

A cyst is a closed sac having a distinct biological membrane and cell division on the nearby Biological tissue. It may contain air, fluids, or semi-solid material....
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


Germ cell tumors of children are the subject of clinical research
Clinical research

Clinical research is a branch of medical science that determines the safety and effectiveness of medications, Medical device, diagnosticss, and medical treatment intended for human use....
 by the worldwide Children's Oncology Group
Children's Oncology Group

The Children's Oncology Group is a worldwide clinical trial cooperative group supported by the National Cancer Institute and fashioned with the mission of studying childhood cancers....
 (COG), in a number of studies coordinated by Dr. John Cullen, MD.

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
Late effect

In medicine, a late effect is a condition that appears after the acute phase of an earlier, causal condition has run its course. A late effect can be caused directly by the earlier condition, or indirectly by the treatment for the earlier condition....
s of treatment.

See also

  • Embryonic stem cells


External links

  • humpath (Pathology images)