 | This article has multiple issues. Please help improve it or discuss these issues on the talk page . (Learn how and when to remove these template messages)
|
Metanephric dysplastic hematoma of the sacral region (MDHSR) has been described by Cozzutto and Lazzaroni-Fossati in 1980, [1] by Posalaki et al. in 1981 [2] and by Cozzutto et al. in 1982. [3] Three additional cases were seen by Finegold. [4]
The case reported by Cozzutto and Lazzaroni-Fossati involved a premature male newborn with bilateral renal dysplasia and a sacrococcygeal mass featuring a histological picture of renal dysplasia. The case reported by Cozzutto et al. and those studied by Finegold featured changes of renal dysplasia including immature tubules surrounded by a collarette of cellular mesenchyme, glomeruloid figures, tubules and nests of cartilage in a background of adipose tissue and fibrous tissue where muscle fibres, nerve bundles, and calcospherites were also seen.
The two cases reported by Posalaki et al. showed blastema, glomeruli, and tubuli. In several cases the mass was removed during reparative surgery for meningocele or myelomeningocele.
Alston et al. described immature renal tissue in a lumbosacral subcutaneous lipoma with intradural extension in a 6-day-old female. [5] Horestein et al. reported a lumbosacral nephrogenic rest including blastema (mature tubules and nephrons) in abundant stroma unassociated to spinal dysraphism. [6] The case reported by Ibrahim et al. in a 2-year-old boy comprised adipose and fibrous tissue with neuroglial elements, striated muscle fibres and clusters of glomeruloid structures and immature tubules. [7]
Sacrococcygeal teratoma with nephroblastoma was reported by Ward and Dehner and by Trebbi et al. [8] [9] Cases of primary lumbosacral Wilms tumor were described by Abrahams et al. and by Govender et al. [10] [11]
It is therefore of primary importance the distinction of MDHSR from sacrococcygeal teratoma and from primary lumbosacral Wilms tumor.
A teratoma is a tumor made up of several different types of tissue, such as hair, muscle, teeth, or bone. Teratomata typically form in the tailbone, ovary, or testicle.
Wilms' tumor or Wilms tumor, also known as nephroblastoma, is a cancer of the kidneys that typically occurs in children, and occurs most commonly as a renal tumor in child patients. It is named after Max Wilms, the German surgeon (1867–1918) who first described it.
A dermoid cyst is a teratoma of a cystic nature that contains an array of developmentally mature, solid tissues. It frequently consists of skin, hair follicles, and sweat glands, while other commonly found components include clumps of long hair, pockets of sebum, blood, fat, bone, nail, teeth, eyes, cartilage, and thyroid tissue.
An ependymoma is a tumor that arises from the ependyma, a tissue of the central nervous system. Usually, in pediatric cases the location is intracranial, while in adults it is spinal. The common location of intracranial ependymomas is the fourth ventricle. Rarely, ependymomas can occur in the pelvic cavity.
Sacrococcygeal teratoma (SCT) is a type of tumor known as a teratoma that develops at the base of the coccyx (tailbone) and is thought to be primarily derived from remnants of the primitive streak. Sacrococcygeal teratomas are benign 75% of the time, malignant 12% of the time, and the remainder are considered "immature teratomas" that share benign and malignant features. Benign sacrococcygeal teratomas are more likely to develop in younger children who are less than 5 months old, and older children are more likely to develop malignant sacrococcygeal teratomas.
Intermediate mesoderm or intermediate mesenchyme is a narrow section of the mesoderm located between the paraxial mesoderm and the lateral plate of the developing embryo. The intermediate mesoderm develops into vital parts of the urogenital system.
Denys–Drash syndrome (DDS) or Drash syndrome is a rare disorder or syndrome characterized by gonadal dysgenesis, nephropathy, and Wilms' tumor.
Malignant rhabdoid tumour (MRT) is a very aggressive form of tumour originally described as a variant of Wilms' tumour, which is primarily a kidney tumour that occurs mainly in children.
Currarino syndrome is an inherited congenital disorder where either the sacrum is not formed properly, or there is a mass in the presacral space in front of the sacrum, and there are malformations of the anus or rectum. It occurs in approximately 1 in 100,000 people.
Ectomesenchymoma is a rare, fast-growing tumor of the nervous system or soft tissue that occurs mainly in children, although cases have been reported in patients up to age 60. Ectomesenchymomas may form in the head and neck, abdomen, perineum, scrotum, or limbs. Also called malignant ectomesenchymoma.
An immature teratoma is a teratoma that contains anaplastic immature elements, and is often synonymous with malignant teratoma. A teratoma is a tumor of germ cell origin, containing tissues from more than one germ cell line, It can be ovarian or testicular in its origin. and are almost always benign. An immature teratoma is thus a very rare tumor, representing 1% of all teratomas, 1% of all ovarian cancers, and 35.6% of malignant ovarian germ cell tumors. It displays a specific age of incidence, occurring most frequently in the first two decades of life and almost never after menopause. Unlike a mature cystic teratoma, an immature teratoma contains immature or embryonic structures. It can coexist with mature cystic teratomas and can constitute of a combination of both adult and embryonic tissue. The most common symptoms noted are abdominal distension and masses. Prognosis and treatment options vary and largely depend on grade, stage and karyotype of the tumor itself.
A ureteral neoplasm is a type of tumor that can be primary, or associated with a metastasis from another site.
Juxtaglomerular cell tumor is an extremely rare kidney tumour of the juxtaglomerular cells, with fewer than 100 cases reported in literature. This tumor typically secretes renin, hence the former name of reninoma. It often causes severe hypertension that is difficult to control, in adults and children, although among causes of secondary hypertension it is rare. It develops most commonly in young adults, but can be diagnosed much later in life. It is generally considered benign, but its malignant potential is uncertain.
Metanephric adenoma (MA) is a rare, benign tumour of the kidney, that can have a microscopic appearance similar to a nephroblastoma, or a papillary renal cell carcinoma.
Perlman syndrome (PS), also known as nephroblastomatosis-fetal ascites-macrosomia-Wilms tumor syndrome, is a rare overgrowth syndrome caused by autosomal recessive mutations in the DIS3L2 gene. PS is characterized by macrocephaly, neonatal macrosomia, nephromegaly, renal dysplasia, dysmorphic facial features, and increased risk for Wilms' tumor. The syndrome is associated with high neonatal mortality.
Scrotalultrasound is a medical ultrasound examination of the scrotum. It is used in the evaluation of testicular pain, and can help identify solid masses.
Pleomorphic anaplastic neuroblastoma (PAN) is a striking aspect of neuroblastoma first described by Cozzutto and Carbone in 1988. Another case was thereafter reported by Cowan, et al. with cytogenetic and immunohistological analysis in a 28-year-old man. The case described by Navarro, et al. showed MYCN amplification and a 1p36 deletion as measured with FISH in 13% of cells. Additionally there was a main cell population with a DNA index of 2 indicating a tetraploid DNA content and a high expression of MIBI (Ki-67), bel 2, p53, and P-glycoprotein, either correlated with rapid progression of disease.
Nephrogenic adenoma is a benign growth typically found in the urinary bladder.
The xanthogranulomatous process (XP), is a form of acute and chronic inflammation characterized by an exuberant clustering of foamy macrophages among other inflammatory cells. Localization in the kidney and renal pelvis has been the most frequent and better known occurrence followed by that in the gallbladder but many others have been subsequently recorded. The pathological findings of the process and etiopathogenetic and clinical observations have been reviewed by Cozzutto and Carbone.
Renal anaplastic sarcoma is a rare tumour of the kidney. By 2017 about 25 cases have been reported. This tumour occurs in children and young adults and is more common in females than males.
