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Typical signs and symptoms of Wilms tumor include the following:
- a painless, palpable abdominal mass
- loss of appetite
- abdominal pain
- fever
- nausea and vomiting
- blood in the urine (in about 20% of cases)
- high blood pressure in some cases (especially if synchronous or metachronous bilateral kidney involvement)
Individuals presenting with fibrosarcoma are usually adults aged thirty to fifty five years, often presenting with pain. In adults, males have a higher incidence for fibrosarcoma than females.
Lymphoepithelioma-like carcinoma (LELC) is a medical term referring to a histological variant of malignant tumor arising from the uncontrolled mitosis of transformed cells originating in epithelial tissue (or in cells that display epithelial characteristics) that bear microscopic resemblance to lymphoepithelioma (nasopharyngeal carcinoma).
There is considerable variation in the classification of LELC—while it is perhaps most commonly considered a subtype of squamous cell carcinoma, it can also be classified as a form of large cell carcinoma (i.e. when occurring in the lung), and can be considered as a separate, unique entity.
In most anatomical sites, many cases are associated with the Epstein-Barr virus.
In the breast, the macroscopic, microscopic, epidemiologic, and prognostic features of LELC are very similar to "medullary carcinoma"; EBV status is one differentiator.
The tumor may present different degrees of differentiation: low grade (differentiated), intermediate malignancy and high malignancy (anaplastic). Depending on this differentiation, tumour cells may resemble mature fibroblasts (spindle-shaped), secreting collagen, with rare mitoses. These cells are arranged in short fascicles which split and merge, giving the appearance of "fish bone" known as a herringbone pattern. Poorly differentiated tumors consist in more atypical cells, pleomorphic, giant cells, multinucleated, numerous atypical mitoses and reduced collagen production. Presence of immature blood vessels (sarcomatous vessels lacking endothelial cells) favors the bloodstream metastasizing. There are many tumors in the differential diagnosis, including spindle cell melanoma, spindle cell squamous cell carcinoma, synovial sarcoma, leiomyosarcoma, malignant peripheral nerve sheath tumor and biphenotypic sinonasal sarcoma.
The tumor largely affects children under 15 years of age and about 20% only are found in adults with nearly 60% involving males and 40% females (1). The most frequent locations are head and neck (orbit and nasopharynx), central nervous system, abdomen and retroperitoneum, pelvis, perineum, scrotum and prostate(1). Clinical symptoms are not specific and usually caused by local tumor compression and infiltration.
Wilms tumor, also known as nephroblastoma, is a cancer of the kidneys that typically occurs in children, rarely in adults. It is named after Dr. Max Wilms, the German surgeon (1867–1918) who first described it.
Approximately 500 cases are diagnosed in the U.S. annually. The majority (75%) occur in otherwise normal children; a minority (25%) are associated with other developmental abnormalities. It is highly responsive to treatment, with about 90% of patients surviving at least five years.
Angiomyofibroblastoma is an uncommon benign mesenchymal tumor. It occurs almost in the vulvovaginal area of women, but can also be observed in men.
The gross features of AMFB are well-circumscribed. Usually, most tumors grow slowly, and patients do not feel pain. It also has low tendency for local recurrence.
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.
Malignant ectomesenchymoma (MEM) is a rare tumor of soft tissues or the CNS, which is composed of both neuroectodermal elements [represented by ganglion cells and/or well-differentiated or poorly differentiated neuroblastic cells such as ganglioneuroma, ganglioneuroblastoma, neuroblastoma, peripheral primitive neuroectodermal tumors – PNET] and one or more mesenchymal neoplastic elements, usually rhabdomyosarcoma . The most accepted theory suggests that this tumor arises from remnants of migratory neural crest cells and thus from the ectomesenchyme.
Fibroepithelial neoplasms (or tumors) are biphasic tumors. This means they consist of epithelial tissue, and stromal or mesenchymal tissue. They may be benign or malignant.
Examples include:
- Brenner tumor of the Ovary
- Fibroadenoma of the Breast
- Phyllodes tumor of the Breast
Hemangioendothelioma is used to describe a group of vascular neoplasms that may be considered benign as well as malignant, depending on the specific group member's activity.
There are few early warning signs that a patient has a DSRCT. Patients are often young and healthy as the tumors grow and spread uninhibited within the abdominal cavity. These are rare tumors and symptoms are often misdiagnosed by physicians. The abdominal masses can grow to enormous size before being noticed by the patient. The tumors can be felt as hard, round masses by palpating the abdomen.
First symptoms of the disease often include abdominal distention, abdominal mass, abdominal or back pain, gastrointestinal obstruction, lack of appetite, ascites, anemia, and/or cachexia.
Other reported symptoms include unknown lumps, thyroid conditions, hormonal conditions, blood clotting, kidney or urological problems, testicle, breast, uterine, vaginal, or ovarian masses.
ASPS may exist in the patient’s body for a long time before being diagnosed. It can grow large and push aside surrounding tissues for a long time before causing any discomfort. Therefore, ASPS symptoms may either be a painless swelling, or a soreness caused by compressed nerves or muscles, affecting the range of motion in the area.
Alveolar rhabdomyosarcoma (ARMS) is a sub-type of the rhabdomyosarcoma soft tissue cancer family whose lineage is from mesenchymal cells and are related to skeletal muscle cells. ARMS tumors resemble the alveoli tissue that can be found in the lungs. Tumor location varies from patient to patient, but is commonly found in the head and neck region, male and female urogenital tracts, the torso, and extremities. Two fusion proteins can be associated with ARMS, but are not necessary, PAX3-FKHR (now known as FOXO1). and PAX7-FKHR. In children and adolescents ARMS accounts for about 1 percent of all malignancies, has an incidence rate of 1 per million, and most cases occur sporadically with no genetic predisposition.
Solitary fibrous tumor (SFT), also known as fibrous tumor of the pleura, is a rare mesenchymal tumor originating in the pleura or at virtually any site in the soft tissue including seminal vesicle. Approximately 78% to 88% of SFT's are benign and 12% to 22% are malignant.
Vascular tissue neoplasms, like neoplasms of all tissues, are classified to benign and malignant ones, according to their biological behavior.
A vascular tissue neoplasm is a tumor arising from endothelial cells, the cells that line the wall of blood vessels and lymphatic vessels, as well as the heart. Vascular tissue neoplasms is a group containing tumors with the same tissue origin; in other words, it denotes histological classification, rather than anatomic (i.e. where in the body the neoplasm is found) or clinical one. They can occur everywhere in the body where vessels are to be found.
Although ASPS displays a relatively indolent course, the ultimate prognosis is poor and is often characterized by late metastases.
Desmoplastic small-round-cell tumor is an aggressive and rare cancer that primarily occurs as masses in the abdomen. Other areas affected may include the lymph nodes, the lining of the abdomen, diaphragm, spleen, liver, chest wall, skull, spinal cord, large intestine, small intestine, bladder, brain, lungs, testicles, ovaries, and the pelvis. Reported sites of metastatic spread include the liver, lungs, lymph nodes, brain, skull, and bones.
The tumor is classified as a soft tissue sarcoma. It is considered a childhood cancer that predominantly strikes boys and young adults. The disease rarely occurs in females, but when it does the tumors can be mistaken for ovarian cancer.
In dogs, mast cell tumors are the most frequent round cell tumor.
Microscopical examination shows abundant thin-walled blood vessels with hypocellular and hypercellular areas.
About 80% of pleural SFTs originate in the visceral pleura, while 20% arise from parietal pleura. Although they are often very large tumors (up to 40 cm. in diameter), over half are asymptomatic at diagnosis. While some researchers have proposed that a SFT occupying at least 40% of the affected hemithorax be considered a "giant solitary fibrous tumor", no such "giant" variant has yet been recognized within the most widely used pleural tumor classification scheme.
Some SFTs are associated with the paraneoplastic Doege–Potter syndrome, which is caused by tumor production of IGF-2.
JCT is morphologically characterized by multiple foci malignant mesenchymal epithelioid cells with, often with admixed necrosis, and a perivascular growth pattern. The immunophenotype is rather characteristic, as the neoplastic cells express renin, CD34, smooth muscle actin, CD138, vimentin, collagen IV and is negative for cytokeratins as well as for S100, c-Kit and desmin.
Hemangioendotheliomas may be classified as:
- "Epithelioid sarcoma-like hemangioendothelioma" is an exceedingly rare vascular tumor of intermediate grade that was first described by Steven Billings, Andrew Folpe, and Sharon Weiss in 2003. These tumors are so named because their histologic appearance is very similar to that of epithelioid sarcoma, a more malignant tumor with which they are commonly mistaken.
- "Composite hemangioendothelioma" is a low-grade angiosarcoma typically occurring in adults, although it has been described in infancy.
- "Spindle-cell hemangioendothelioma") is a vascular tumor that was first described in 1986 by Sharon Weiss, M.D., and commonly presents in a child or young adult who develops blue nodules of firm consistency on a distal extremity. These tumors were reclassified by Dr. Weiss in 1996 as "spindle cell hemangioma", rather than hemangioendothelioma, due to the excellent prognosis observed in a group of 78 patients.
- "Retiform hemangioendothelioma" (also known as a "Hobnail hemangioendothelioma") is a low-grade angiosarcoma, first described in 1994, presenting as a slow-growing exophytic mass, dermal plaque, or subcutaneous nodule.
- "Kaposiform hemangioendothelioma" (also known as "Infantile kaposiform hemangioendothelioma") is an uncommon vascular tumor, first described by Niedt, Greco, et al. (Hemangioma with Kaposi's sarcoma-like features: report of two cases.(Niedt GW, Greco MA, Wieczorek R, Blanc WA, Knowles DM 2nd. that affects infants and young children, with rare cases having also been reported in adults.Pediatr Pathol. 1989;9(5):567-75.)
- "Endovascular papillary angioendothelioma", also known as "Dabska tumor", "papillary intralymphatic angioendothelioma" (PILA), "Dabska-type hemangioendothelioma", "hobnail hemangioendothelioma", and "malignant endovascular papillary angioendothelioma", is a rare low-grade angiosarcoma of lymphatic channels. Approximately 30 such tumors have been described in the medical literature. Although included in the World Health Organization tumor classification, there is uncertainty as to whether EPA is a distinct entity or a heterogenous group of tumours. The lesion usually presents as a slow-growing tumor of the skin and subcutaneous tissues of the head, neck, or extremity, of infants or young children. However, EPA has involved the testicle, deep muscle tissue as a neoplastic transformation of a larger existing benign cavernous hemangioma, bone and spleen, and has been found in adults. Some reports indicate a good prognosis but metastasis is occasionally seen.
- "Infantile hemangioendothelioma" is a rare benign vascular tumour arising from mesenchymal tissue and is usually located in the liver. It often presents in infancy with cardiac failure because of extensive arteriovenous shunting within the lesion. It is the third most common liver tumor in children, the most common benign vascular tumor of the liver in infancy, and the most common symptomatic liver tumor during the first 6 months of life. These hemangioendotheliomas have 2 growth phases: an initial rapid growth phase, which is followed by a period of spontaneous involution (usually within the first 12 to 18 months of life). Detection of the hemangioendothelioma within the first 6 months of life is attributed to the initial rapid growth during this time; however, the tumor has been detected with fetal ultrasonography. Histopathologically, there are 2 types of hepatic hemangioendotheliomas:
- Type I: Hemagioendotheliomas of this type have multiple vascular channels that are formed by an immature endothelial lining with stromal separation from bile ductules.
- Type II: These hemangioendotheliomas have an appearance that is more disorganized and hypercellular, and there are no bile ductules.
Carcinosarcomas are malignant tumors that consist of a mixture of carcinoma (or epithelial cancer) and sarcoma (or mesenchymal/connective tissue cancer). Carcinosarcomas are rare tumors, and can arise in diverse organs, such as the skin, salivary glands, lungs, the esophagus, pancreas, colon, uterus and ovaries.
Four main hypotheses have been proposed for the cellular origins of carcinosarcoma, based largely on the pathology of the disease. First, the collision tumor hypothesis, which proposes the collision of two independent tumors resulting in a single neoplasm, based on the observation that skin cancers and superficial malignant fibrous histiocytomas are commonly seen in patients with sun-damaged skin; second, the composition hypothesis, which suggests that the mesenchymal component represents a pseudosarcomatous reaction to the epithelial malignancy; third, the combination hypothesis, which suggests that both the epithelial and mesenchymal components of the tumor arise from a common pluripotential stem cell that undergoes divergent differentiation; and fourth, the conversion/divergence hypothesis, which argues that the sarcomatous component of the tumor represents a metaplastic sarcomatous transformation of the epithelial component. Despite the remaining uncertainty on the mechanisms that generate these tumors, recent immunohistochemical, ultrastructural, and molecular genetic studies suggest and favor the notion of monoclonality in carcinosarcoma. In addition, identical p53 and KRAS mutations have been identified in both epithelial and mesenchymal components of carcinosarcoma, findings that suggest an early alteration in the histogenesis of the tumor with late transformation or degeneration of the epithelial component into the sarcomatous component.
By hypersecretion of renin, JCT causes hypertension, often severe and usually sustained but occasionally paroxysmal, and secondary hyperaldosteronism inducing hypokalemia, though the later can be mild despite high renin. Both of these conditions may be corrected by surgical removal of the tumor. Asymptomatic cases have been reported.
Polyembryoma is a rare, very aggressive form of germ cell tumor usually found in the ovaries. Polyembryoma has features of both yolk sac tumour and undifferentiated teratoma/embryonal carcinoma, with a characteristic finding of embryoid bodies lying in a loose mesenchymal stroma.
It has been found in association with Klinefelter syndrome.