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Cystic nephromas are diagnosed by biopsy or excision. It is important to correctly diagnose them as, radiologically, they may mimic the appearance of a renal cell carcinoma that is cystic.
As metanephric adenomas are considered benign, they can be left in place, i.e. no treatment is needed.
They generally have a good prognosis. In one larger study, the 5-year and 10-year survival were over 90% and 80% respectively.
Serous cystic neoplasms can come to clinical attention in a variety of ways. The most common symptoms are very non-specific and include abdominal pain, nausea and vomiting. In contrast to many of the other tumors of the pancreas, patients rarely develop jaundice (a yellowing of the skin and eyes caused by obstruction of the bile duct), or weight loss. These signs and symptoms are not specific for a serous cystic neoplasm, making it more difficult to establish a diagnosis. Doctors will therefore often order additional tests.
Once a doctor has reason to believe that a patient may have serous cystic neoplasm, he or she can confirm that suspicion using one of a number of imaging techniques. These include computerized tomography (CT), endoscopic ultrasound (EUS), and magnetic resonance cholangiopancreatography (MRCP). These tests will reveal a cystic mass within the pancreas. The cysts do not communicate with the larger pancreatic ducts. In some cases a fine needle aspiration (FNA) biopsy can be obtained to confirm the diagnosis. Fine needle aspiration biopsy can be performed through an endoscope at the time of endoscopic ultrasound, or it can be performed through the skin using a needle guided by ultrasound or CT scanning.
A growing number of patients are now being diagnosed before they develop symptoms (asymptomatic patients). In these cases, the lesion in the pancreas is discovered accidentally (by chance) when the patient is being scanned (x-rayed) for another reason.
Diagnosis is usually made by ultrasonography showing a solid ovarian lesion, or, on some occasions, mixed tumors with solid and cystic components. Computed tomography and magnetic resonance imaging can also be used to diagnose fibromas.
In a series of 16 patients, 5 (28%) showed elevated levels of CA-125.
Metanephric adenoma is diagnosed histologically. The tumours can be located at upper pole, lower pole and mid-hilar region of the kidney; they are well circumscribed but unencapsulated, tan pink, with possible cystic and hemorrhagic foci. They show a uniform architecture of closely packed acinar or tubular structures of mature and bland appearance with scanty interposed stroma. Cells are small with dark staining nuclei and inconspicuous nucleoli. Blastema is absent whereas calcospherites may be present. Glomeruloid figures are a striking finding, reminiscent of early fetal metenephric tissue. The lumen of the acini may contain otherwise epithelial infoldings or fibrillary material but it is quite often empty. Mitoses are conspicuously absent.
In the series reported by Jones "et al." tumour cells were reactive for Leu7 in 3 cases of 5, to vimentine in 4 of 6, to cytocheratin in 2 of 6, to epithelial membrane antigen in 1 of 6 cases and muscle specific antigen in 1 of 6.
Olgac "et al." found that intense and diffuse immunoreactivity for alpha-methylacyl-CoA racemase (AMACR) is useful in differentiating renal cell carcinoma from MA but a panel including AMACR, CK7 and CD57 is better in this differential diagnosis.
Differential diagnosis may be quite difficult indeed as exemplified by the three malignancies initially diagnosed as MA that later metastasized, in the report by Pins et al.
EMCas are diagnosed by examination of tissue, e.g. a biopsy.
Its appearance is very similar to adenomyoepithelioma of the breast, which may be the same tumour at a different anatomical site.
The histologic differential diagnosis includes adenoid cystic carcinoma and pleomorphic adenoma.
They are benign lesions and malignant degeneration is rare. They are usually treated with curettage which however have a high recurrence rate of 25%. As such if an en-bloc resection is possible this is advisable
Usually the lesion is surgically removed. Primarily, there is concern that the lesion identified in a patient could be cancerous, but there is also the risk of torsion, and possibly the development of symptoms. A stable lesion, however, could be clinically followed.
Ultrasonography of liver tumors involves two stages: detection and characterization. Tumor detection is based on the performance of the method and should include morphometric information (three axes dimensions, volume) and topographic information (number, location specifying liver segment and lobe/lobes). The specification of these data is important for staging liver tumors and prognosis. Tumor characterization is a complex process based on a sum of criteria leading towards tumor nature definition. Often, other diagnostic procedures, especially interventional ones are no longer necessary. Tumor characterization using the ultrasound method will be based on the following elements: consistency (solid, liquid, mixed), echogenicity, structure appearance (homogeneous or heterogeneous), delineation from adjacent liver parenchyma (capsular, imprecise), elasticity, posterior acoustic enhancement effect, the relation with neighboring organs or structures (displacement, invasion), vasculature (presence and characteristics on Doppler ultrasonography and contrast-enhanced ultrasound (CEUS).
The characteristics of cystic nephromas are:
- Cysts lined by a simple epithelium with a "hobnail morphology", i.e. the nuclei of the cyst lining epithelium bulges into the lumen of the cysts,
- Ovarian-like stroma that has a:
- Spindle cell morphology, and has a
- Basophilic cytoplasm.
Cystic nephromas have an immunostaining pattern like ovarian stroma; they are positive for:
- Estrogen receptor (ER),
- Progesterone receptor (PR) and
- CD10.
A fibroadenoma is usually diagnosed through clinical examination, ultrasound or mammography, and often a needle biopsy sample of the lump.
These lesions rarely require surgery unless they are symptomatic or the diagnosis is in question. Since these lesions do not have malignant potential, long-term observation is unnecessary. Surgery can include the removal of the head of the pancreas (a pancreaticoduodenectomy), removal of the body and tail of the pancreas (a distal pancreatectomy), or rarely removal of the entire pancreas (a total pancreatectomy). In selected cases the surgery can be performed using minimally invasive techniques such as laparoscopy.
The diagnosis is based on tissue examination, e.g. biopsy.
Under the microscope, glassy cell carcinoma tumours are composed of cells with a glass-like cytoplasm, typically associated with an inflammatory infiltrate abundant in eosinophils and very mitotically active. PAS staining distinctively extenuates the plasma membrane.
Upon discovery of a liver tumor, the main issue in the workup is to determine whether the tumor is benign or malignant. Many imaging modalities are used to aid in the diagnosis of malignant liver tumors. For the most common of these, hepatocellular carcinoma (HCC), these include sonography (ultrasound), computed tomography (CT) and magnetic resonance imaging (MRI). When imaging the liver with ultrasound, a mass greater than 2 cm has more than 95% chance of being HCC. The majority of cholangiocarcimas occur in the hilar region of the liver, and often present as bile duct obstruction. If the cause of obstruction is suspected to be malignant, endoscopic retrograde cholangiopancreatography (ERCP), ultrasound, CT, MRI and magnetic resonance cholangiopancreatography (MRCP) are used.
Tumor markers, chemicals sometimes found in the blood of people with cancer, can be helpful in diagnosing and monitoring the course of liver cancers. High levels of alpha-fetoprotein (AFP) in the blood can be found in many cases of HCC and intrahepatic cholangiocarcinoma. Cholangiocarcinoma can be detected with these commonly used tumor markers: carbohydrate antigen 19-9 (CA 19-9), carcinoembryonic antigen (CEA) and cancer antigen 125 (CA125). These tumour markers are found in primary liver cancers, as well as in other cancers and certain other disorders..
Carcinoma ex pleomorphic adenoma is diagnosed by examining it under the microscope with consideration of the individual history.
In approximately 75% of cases ca ex PAs arise in a pleomorphic adenoma that is apparent when the tumour is excised. In the other approximately 25% of cases the individual had a pleomorphic adenoma excised previously and the diagnosis is made based on (1) the presence of a carcinoma, and (2) the history of a pleomorphic adenoma at that location.
Littoral cell angiomas show in CT scans. They are diagnosed by pathologists by taking a sample of the tumour via Fine Needle Aspiration or Core Needle Aspiration or from a splenectomy. Histologically, they have anastoming small vascular channels and cystic spaces with papillary projections.
Regardless of location, all rhabdoid tumours are highly aggressive, have a poor prognosis, and tend to occur in children less than two years of age.
Imaging studies such as Computerized Tomography (CT) and Magnetic Resonance Imaging (MRI) can aid diagnosis. Medulloepithelioma appears isodense or hypodense with variable heterogeneity and calcification on non-contrast CT scan, and enhances with contrast. This radiographical finding is consistent with a primitive neuroectodermal tumour, especially in children. Blood studies and imaging studies of the abdomen may be used to detect metastases.
Needle aspiration biopsy can be used to aid diagnosis. Definitive diagnosis requires histopathological examination of surgically excised tumour tissues.
Histologically, medulloepithelioma resemble a primitive neural tube and with neuronal, glial and mesenchymal elements. Flexner-Wintersteiner rosettes may also be observed.
Immunohistochemically, neural tube-like structures are vimentin positive in the majority of medulloepitheliomas. Poorly differentiated medulloepitheliomas are vimentin negative.
Plain film
often seen as a lobulated, eccentric radiolucent lesion
long axis parallel to long axis of long bone
no periosteal reaction (unless a complicating fracture present)
geographic bone destruction: almost 100%
well defined sclerotic margin: 86%
there can be presence of septations (pseudotrabeculation): 57% 2
there can be presence of matrix calcification in a small proportion of cases: 12.5%1
MRI
MR features are often not particularly specific. Signal characteristics include
T1 - low signal
T1 C+ (Gd) -
the majority (~70%) tend to show peripheral nodular enhancement
~ 30% diffuse contrast enhancement and this can be either homogeneous or heterogeneous 19
T2 - high signal
Bone scan
A scintigraphic "doughnut sign" has been described in this tumour type 11. However, this is very non-specific and can be found in a plethora of other bone lesions.
Renal medullary fibromas are diagnosed by pathologists based on the examination of tissue.
They consist of bland spindle-shaped or stellate-shaped cells in a loose stroma. Renal tubules may be entrapped.
On ultrasound, a sertoli cell tumour appears as a hypoechoic intratesticular lesion which is usually solitary. However, the large cell subtype might present as multiple and bilateral masses with large areas of calcification. An MRI may also be conducted, but this typically is undefinitive.
Microscopy and immunohistochemistry are the only way to give a definitive diagnosis, especially when there is a suspected seminoma.
The criteria for diagnosing BACs have changed since 1999. Under the new definition, BAC is defined as a tumor that grows in a lepidic (that is, a scaly covering) fashion along pre-existing airway structures, without detectable invasion or destruction of the underlying tissue, blood vessels, or lymphatics. Because invasion must be ruled out, BAC can be diagnosed only after complete sectioning and examination of the entire tumor, not using biopsy or cytology samples. BAC is considered a pre-invasive malignant lesion that, after further mutation and progression, eventually generates an invasive adenocarcinoma. Therefore, it is considered a form of carcinoma "in situ" (CIS).
The FDA has approved cryoablation of a fibroadenoma as a safe, effective and minimally-invasive alternative to open surgical removal in 2001. In the procedure, ultrasound imaging is used to guide a probe into the mass of breast tissue. Extremely cold temperatures are then used to destroy the abnormal cells, and over time the cells are reabsorbed into the body. The procedure can be performed as an outpatient surgery using local anesthesia only, and leaves substantially less scarring than open surgical procedures and no breast tissue deformation.
The American Society of Breast Surgeons recommends the following criteria to establish a patient as a candidate for cryoablation of a fibroadenoma:
1. The lesion must be sonographically visible.
2. The diagnosis of a fibroadenoma must be confirmed histologically.
3. The lesion should be less than 4 cm in diameter.
The treatment of a Pancoast lung cancer may differ from that of other types of non-small cell lung cancer. Its position and close proximity to vital structures (such as nerves and spine) may make surgery difficult. As a result, and depending on the stage of the cancer, treatment may involve radiation and chemotherapy given prior to surgery (neoadjuvant treatment).
Surgery may consist of the removal of the upper lobe of a lung together with its associated structures (subclavian artery, vein, branches of the brachial plexus, ribs and vertebral bodies), as well as mediastinal lymphadenectomy. Surgical access may be via thoracotomy from the back or the front of the chest and modifications