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The term "duct ectasia syndrome" has been used to describe symptoms of nonpuerperal mastitis, possibly associated with nipple inversion and nipple discharge. In some contexts, it was used to describe a particular form of nonpuerperal mastitis coincident with fibrocystic disease, frequently involving pasty (coloured) nipple discharge, nipple retraction, retroareolar abscess and blue dome cysts. Abscessation is not very frequent but by some definitions recurrent subareolar abscess is merely a variant of duct ectasia syndrome - abscessation would be obviously more frequent with this definition.
Duct ectasia syndrome has been associated with histopathological findings that are distinct from a simple duct widening. In addition to nonspecific duct widening the myoepithelial cell layer is atrophic, missing or replaced by fibrous tissue. The original cuboidal epithelial layer may be also severely impaired or missing. Characteristic calcifications are often visible on mammographic images.
Periductal mastitis, comedo mastitis, secretory disease of the breast, plasma cell mastitis and mastitis obliterans are sometimes considered special cases or synonyms of duct ectasia syndrome.
The condition is usually self-limiting, and thus not indicated for surgery.
Multiple imaging modalities may be necessary to evaluate abnormalities of the nipple-areolar complex.
In two studies performed in Japan, high-resolution MRI with a microscopy coil yielding 0.137-mm in-plane resolution has been used to confirm the presence of abscesses, isolated fistulas and inflammation and to reveal their position in order to guide surgery.
Lesions of the nipple and areola, such as nipple adenoma, may be difficult to image clearly on routine mammogram or ultrasonography. Nipple adenomas can be imaged using magnetic resonance imaging (MRI) and conventional or MR ductogram.
A cyst of Montgomery may be asymptomatic. Yet, a cyst of Montgomery usually is diagnosed when a female patient, 10–20 years of age, complains to a healthcare professional of breast pain (mastalgia), inflammation or a palpable nodule in the breast. The diagnosis is made clinically, when a palpable nodule is felt in the retroareolar area.
The diagnosis can be confirmed with ultrasonography, frequently showing a simple cyst in the retroareolar area. In some patients, multiple cysts or bilateral cysts may exist. Cysts of Montgomery may have liquid content with an echogenic or calcific sediment.
The clinical management of a cyst of Montgomery depends upon the symptoms of the patient.
If there are no signs of infection, a cyst of Montgomery can be observed, because more than 80% resolve spontaneously, over only a few months. However, in some cases, spontaneous resolution may take up two years. In such cases, a repeat ultrasonography may become necessary. If, however, the patient has signs of an infection, for example reddening (erythema), warmth, pain and tenderness, a treatment for mastitis can be initiated, which may include antibiotics and non-steroidal anti-inflammatory drugs (NSAIDs). With treatment, inflammatory changes usually disappear quickly. In rare cases, drainage may become necessary. A surgical treatment of a cyst of Montgomery, i.e. a resection, may become necessary only if a cyst of Montgomery persists, or the diagnosis is questioned clinically.
The prognosis seems to be excellent. In one series, all adolescent patients with a cyst of Montgomery had a favourable outcome.
It is important to include that the lesion is associated with another cancer. A biopsy will establish the diagnosis. The histology of the lesion is the same as for Paget's disease of the breast.
The appropriate treatment in contemporary western medicine is complete surgical excision of the abnormal growth with a small amount of normal surrounding breast tissue.
The diagnosis of hemosuccus pancreaticus can be difficult to make. Most patients who develop bleeding in the gastrointestinal tract have endoscopic procedures done to visualize the bowel in order to find and treat the source of the bleeding. With hemosuccus, the bleeding is coming from the pancreatic duct which enters into the first part of the small intestine, termed the duodenum. Typical gastroscopes used to visualize the esophagus, stomach and duodenum are designed with fiber-optic illumination that is directed in the same direction as the endoscope, meaning that visualization is in the forward direction. However, the pancreatic duct orifice is located on the side of the duodenum, meaning that it can be missed on forward-viewing endoscopy. A side-viewing endoscope (known as a "duodenoscope", or "side-viewer") used for endoscopic retrograde cholangiopancreatography (ERCP), a procedure to visualize the bile ducts and pancreatic duct on fluoroscopy, can be used to localize the bleeding to the pancreatic duct. It can be confused with bleeding from the common bile duct on endoscopy, leading to the term "pseudohematobilia".
Liver function test is normal apart from an increased serum bilirubin in the event of pancreaticobiliary reflux. Serum amylase is normal outside episodes of acute pancreatitis. It is difficult to diagnose HP because the bleeding is usually intermittent. Endoscopy is essential in ruling out other causes of upper gastrointestinal bleeding and in rare cases; active bleeding can be seen from the duodenal ampulla. Even though endoscopy may be normal, it helps to rule out other causes of upper digestive bleeding (erosive gastritis, peptic ulcers, and oesophageal and gastric fundus varices, etc.). Ultrasonography can be used to visualize pancreatic pseudocysts or aneurysm of the peripancreatic arteries. Doppler ultrasound or dynamic ultrasound has been reported to be diagnostic. Contrast-enhanced CT is an excellent modality for demonstrating the pancreatic pathology and can also demonstrate features of chronic pancreatitis, pseudocysts, and pseudoaneurysms. On precontrast CT, the characteristic finding of clotted blood in the pancreatic duct, known as the sentinel clot, is seldom seen. Computed tomography may show simultaneous opacification of an aneurysmal artery and pseudocyst or persistence of contrast within a pseudocyst after the arterial phase. Again, these findings are only suggestive of the diagnosis. Ultimately, angiography is the diagnostic reference standard. Angiography identifies the causative artery and allows for delineation of the arterial anatomy and therapeutic intervention.
Paget's disease of the vulva, a rare disease, may be a primary lesion or associated with adenocarcinoma originating from local organs such as the Bartholin gland, the urethra, or the rectum and thus be secondary. Patients tend to be postmenopausal.
Paget's disease of the penis may also be primary or secondary, and is even rarer than genital Paget’s disease in women. At least one case has been misdiagnosed as Bowen's disease. Isolated Paget's disease of the penis is extremely rare.
Intraductal papillomas of the breast are benign lesions with an incidence of approximately 2-3% in humans.
Two types of intraductal papillomas are generally distinguished. The central type develops near the nipple. They are usually solitary and often arise in the period nearing menopause. On the other hand, the peripheral type are often multiple papillomas arising at the peripheral breasts, and are usually found in younger women. The peripheral type are associated with a higher risk of malignancy.
They are the most common cause of bloody nipple discharge in women age 20-40 and generally do not show up on mammography due to their small size. They may be detectable on ultrasound. A galactogram is the most definitive test but is somewhat invasive.
The masses are often too small to be palpated or felt. A galactogram is therefore necessary to rule out the lesion.
Excision is sometimes performed. Microdochectomy/microdochotomy (removal of a breast duct) is the treatment of choice.
The diagnosis of nipple discharge will be determined upon an examination by a health provider who will ask questions about symptoms and medical history. Tests that may be done include:
- Prolactin blood test
- Thyroid blood tests
- Head CT scan or MRI to look for pituitary tumor
- Mammography
- Ultrasound of the breast
- Breast biopsy
- Ductography or ductogram: an x-ray with contrast dye injected into the affected milk duct
- Skin biopsy, if Paget disease is a concern
A fibroadenoma is usually diagnosed through clinical examination, ultrasound or mammography, and often a needle biopsy sample of the lump.
Treatment is problematic unless an underlying endocrine disorder can be successfully diagnosed and treated.
A study by Goepel and Panhke provided indications that the inflammation should be controlled by bromocriptine even in absence of hyperprolactinemia.
Antibiotic treatment is given in case of acute inflammation. However, this alone is rarely effective, and the treatment of a subareaolar abscess is primarily surgical. In case of an acute abscess, incision and drainage are performed, followed by antibiotics treatment. However, in contrast to peripheral breast abscess which often resolves after antibiotics and incision and drainage, subareaolar breast abscess has a tendency to recur, often accompanied by the formation of fistulas leading from inflammation area to the skin surface. In many cases, in particular in patients with recurrent subareolar abscess, the excision of the affected lactiferous ducts is indicated, together with the excision of any chronic abscess or fistula. This can be performed using radial or circumareolar incision.
There is no universal agreement on what should be the standard way of treating the condition. In a recent review article, antibiotics treatment, ultrasound evaluation and, if fluid is present, ultrasound-guided fine needle aspiration of the abscess with an 18 gauge needle, under saline lavage until clear, has been suggested as initial line of treatment for breast abscess in puerperal and non-puerperal cases including central (subareolar) abscess (see breast abscess for details). Elsewhere, it has been stated that treatment of subareolar abscess is unlikely to work if it does not address the ducts as such.
Duct resection has been traditionally used to treat the condition; the original Hadfield procedure has been improved many times but long term success rate remains poor even for radical surgery. Petersen even suggests that damage caused by previous surgery is a frequent cause of subareolar abscesses. Goepel and Pahnke and other authors recommend performing surgeries only with concomitant bromocriptine treatment.
Treatment of hemosuccus pancreaticus depends on the source of the hemorrhage. If the bleeding is identified on angiography to be coming from a vessel that is small enough to occlude, embolization through angiography may stop the bleeding. Both coils in the end-artery and stents across the area of bleeding have been used to control the hemorrhage. However, the bleeding may be refractory to the embolization, which would necessitate surgery to remove the pancreas at the source of hemorrhage. Also, the cause of bleeding may be too diffuse to be treated with embolization (such as with pancreatitis or with pancreatic cancer). This may also require surgical therapy, and usually a distal pancreatectomy, or removal of the part of the pancreas from the area of bleeding to the tail, is required.
The original report was of a family in Cardiff, United Kingdom. There are subsequent reports of patients from the USA, France, Australia, UAE, India and from Cuba.
Scalp–ear–nipple syndrome (also known as "Finlay–Marks syndrome") is a condition associated with aplasia cutis congenita.
PUNLMPs are exophytic lesions that appear friable to the naked eye and when imaged during cystoscopy.
They are definitively diagnosed after removal by microscopic examination by pathologists.
Histologically, they have a papillary architecture with slender fibrovascular cores and rare basal mitoses. The papillae rarely fuse and uncommonly branch. Cytologically, they have uniform nuclear enlargement.
They cannot be reliably differentiated from low grade papillary urothelial carcinomas using cytology, and their diagnosis (vis-a-vis low grade papillary urothelial carcinoma) has a poor inter-rater reliability.
Pathologic grading and staging tumors are:
graded by the degree of cellular atypia (G1->G3), and
staged:
Recommended tests are a mammogram and a biopsy to confirm the diagnosis, and cytopathology may also be helpful. Paget's disease is difficult to diagnose due to its resemblance to dermatitis and eczema; even in patients after ductal carcinoma in situ surgery. Eczema tends to affect the areola first, and then the nipple, whereas Paget's spreads from the nipple.
During a physical examination, the doctor examines the unusual areas of the breast, especially the appearance of the skin on and around the nipples and feeling for any lumps or areas of thickening.
The most common test used to diagnose Paget's disease is the biopsy, removal of a tissue sample from the affected area which is then examined under the microscope by a pathologist, who distinguishes Paget cells from other cell types by staining tissues to identify specific cells (immunohistochemistry). Samples of nipple discharge may also be examined under the microscope to determine whether Paget cells are present.
Imprint or scrape cytopathology may be useful: scraping cells from the affected area, or pressing them onto a glass slide to be examined under the microscope.
On average, a woman may experience signs and symptoms for six to eight months before a diagnosis is made.
Note: differentiation is done accurately by microscopic examination only.
Diagnostic techniques:
- antibodies (IgG) precipitates complement (C3) in the lamina lucida of the basement membrane.
- Circulating auto-antibodies to BP-1 antigen (located in hemidesmosome). 50% have BP-2.
- Positive Nikolsky sign.
- IgG, C3 deposition at BM creating smooth line in immunofluorescent analysis.
The diagnosis is based on tissue examination, e.g. biopsy.
The name of the lesion describes it microscopic appearance. It has nipple-like structures with fibrovascular cores () that are long in relation to their width (villus-like), which are covered with a glandular pseudostratified columnar epithelium.
Ulnar–mammary syndrome or Schinzel syndrome is a cutaneous condition characterized by nipple and breast hypoplasia or aplasia.
It has been associated with TBX3.
Initially, an evaluation of malignancy is always indicated. If no abnormality is found, then a surgical duct excision may resolve the symptoms. Treatment also depends on whether single-duct or multiple-duct discharge is present, and whether the symptoms of nipple discharge are distressing to the patient. In some cases, there may be no need for any further intervention; in others, microdochectomy or a total duct excision may be appropriate. If the patient wishes to conserve the ability to breastfeed and only single-duct discharge is present, then ductoscopy or galactography should be considered in view of performing a localised duct excision. Once the cause of the nipple discharge is found, it will be treated by the health provider who will evaluate:
- Whether changing any medicine that has caused the discharge is appropriate
- Whether any lumps should be removed
- Whether some or all of the breast ducts should be removed
- If a prescription for cream to treat skin changes around the nipple will be helpful
- If medicines to treat a health condition are required
If all the tests are normal, treatment may not be necessary. A follow up mamogram mammogram and physical exam may be prescribed within 1 year.
PUNLMPs are treated like non-invasive low grade papillary urothelial carcinomas, excision and regular follow-up cystoscopies.
There is a rare occurrence of a pelvic recurrence of a low-grade superficial TCC after cystectomy. Delayed presentation with recurrent low-grade urothelial carcinoma is an unusual entity and potential mechanism of traumatic implantation should be considered. Characteristically low-grade tumors are resistant to systemic chemotherapy and curative-intent surgical resection of the tumor should be considered.