I wanted to post something for those of you that follow the blog.
Things have been a little crazy in the Maday household for the past several weeks. We had 3 weekends of traveling all over Tennessee for gymnastics meets for my girls. I am also ramping up my training for a potential Scottish games competition in May and we have been gearing up and preparing for my middle daughter’s Bat Mitzvah in June. And…..tonight is the first night of Passover.
Needless to say, I have not had as much “free time” on the weekends to devout to the PAINE Podcast Project. I hope to get back to the regularly scheduled content (EpPAINEnym, PAINE Pearls, and medical posts). Things should hopefully open back up in the next few weeks, but I will try to do what I can, when I can.
Definition – Raising of the patient’s arms over their head (until the arms touch their face) causes flushing and congestion of head and neck due to venous congestion and thoracic inlet obstruction.
Clinical Significance –this is a simple physical examination maneuver to diagnose a patient with superior vena cava syndrome and pressure on the thoracic inlet. A positive sign is flushing and cyanosis of the head and neck with possible respiratory distress with prolonged holding and is associated with mediastinal masses, goiters, and mediastinal lymphadenopathy.
History – Named after Hugh Spear Pemberton (1890-1956), who was an English physician and recieved his medical doctorate from the University of Liverpool in 1913. He would subsequently serve as a physician in the Royal Medical Corp during World War I and returned to Liverpool at the David Lewis Northern Hospital where he would spend his entire career. He founded one of the first diabetic clinics there in 1922 and made a name for himself in he area of endocrinology and receiving Fellowship in the Royal College of Physicians in 1941. It was in 1946 that he published a very short letter to the Lancet describing his eponymous maneuver.
References
Firkin BG and Whitwirth JA. Dictionary of Medical Eponyms. 2nd ed. New York, NY; Parthenon Publishing Group. 1996.
Bartolucci S, Forbis P. Stedman’s Medical Eponyms. 2nd ed. Baltimore, MD; LWW. 2005.
Yee AJ, Pfiffner P. (2012). Medical Eponyms (Version 1.4.2) [Mobile Application Software]. Retrieved http://itunes.apple.com.
Other Known Aliases – Heinz-Ehrlich bodies, Ehrlich Inner Body
Definition – Deep purple small irregular bodies in red cells stained with crystal violet which represents denatured hemoglobin due to oxidative damage.
Clinical Significance –Seen in conditions with high oxidative stress such as G6PD deficiency, alpha thalassemia, NADPH deficiency, chronic liver disease, and asplenia.
History – Named after Robert Heinz (1865-1924), a German physician and pharmcologist who recieved his medical doctorate from the University of Breslau in 1888. He would work in the university chemical laboratory in Jena and Munich throughout his career studying pathology, inflammation, degeneration, and regeneration of blood. It was during this time, specifically in 1890, that he published a study on the blood of guinea pigs treated with acetylphenylhydrazine to intoduce oxidative inflammation and identified his eponymous cellular structure. Of note, Paul Ehrlich (1854-1915) is also regionally credited with identifying this structure but did not formally publish his findings.
References
Firkin BG and Whitwirth JA. Dictionary of Medical Eponyms. 2nd ed. New York, NY; Parthenon Publishing Group. 1996.
Bartolucci S, Forbis P. Stedman’s Medical Eponyms. 2nd ed. Baltimore, MD; LWW. 2005.
Yee AJ, Pfiffner P. (2012). Medical Eponyms (Version 1.4.2) [Mobile Application Software]. Retrieved http://itunes.apple.com.
R. Heinz. Morphologische Veränderungen des roten Blutkörperchens durch Gifte. [Virchows] Archiv für pathologische Anatomie und Physiologie und für klinische Medizin, Berlin, 1890, 122: 112-116
Definition – Autosomal dominant, hereditary clotting disorder arising from a deficiency in the quantity and/or quality of von Willebrand factor (vWF), which is a protein required for platelet adhesion and involved in primary hemostasis. The genetic defect responsible for vWF production the vWF gene located on the short arm of chromosome 12 (12p13.2)
Clinical Significance –This is the most common type of hereditary blood-clotting disorder in humans, with 3 main hereditary types and multiple subtypes. Type 1 is the most common and often asymptomatic, Type 2 can have mild to moderate symptoms, Type 3 is the most severe and can manifest with hemarthosis and internal bleeding.
History – Named after Erik Adolf von Willebrand (1870-1949), a Finnish physician who received his medical doctorate from the University of Helsinki 1896, and who took a special interest in hematology and coagulation. In 1924, a 5yo girl was brought to him due to a bleeding disorder and he successfully performed a family history map on the girl’s 66 living family members and discovered the autosomal dominant pattern. He published his findings in 1926 in Swedish calling it “pseudo-hemophilia”, but it wasn’t until 1931 (when it was translated into German) did it gain any traction in the medical community.
References
Firkin BG and Whitwirth JA. Dictionary of Medical Eponyms. 2nd ed. New York, NY; Parthenon Publishing Group. 1996.
Bartolucci S, Forbis P. Stedman’s Medical Eponyms. 2nd ed. Baltimore, MD; LWW. 2005.
Yee AJ, Pfiffner P. (2012). Medical Eponyms (Version 1.4.2) [Mobile Application Software]. Retrieved http://itunes.apple.com.
27yo, G1P1001, presents to the OBGYN office with complaints of a “breast lump”. She states she first noticed it during her pregnancy last year, but did not think anything of it until her mother told her to have it checked it out. She denies any personal or family history of malignancies or any change in size since her pregnancy. Visual inspection shows no nipple retraction, skin dimpling, or asymmetry, and physical examination reveals a 1.5cm, well-defined, non-tender mobile mass 4cm from the nipple in the 1 o’clock position.
What would be the next step in the diagnostic evaluation of this lesion?
She is obviously concerned about breast cancer. Are they any concerning features in this presentation?
Answer
Given the ease of palpation, an ultrasound of the breast to further evaluate the lesion is the next appropriate step. Fine needle aspiration (FNA) or biopsy is not warranted in this patient because of her age and the morphology of the lesion.
There are no concerning features in this patient presentation that would raise suspicion of malignancy. < 30 years of age, no nipple retraction, skin dimpling, asymmetry, and a well-defined border on palpation all suggest benignity.
Primary components of the breast are terminal duct lobular ubnits, lobular stroma, interlobular stroma, ducts, and lactiferous sinuses
Epithelium (terminal duct lobular units) is the most hormonally responsive
Natural hormonal changes of puberty, pregnancy, lactation, and menopause can lead to remodeling of these structures
Main Classifications
There are four main classifications of benign breast disorders that are based on the degree of cellular proliferations and atypia present
Nonproliferative
Characterized by acinar dilation and fibrosis
Generally, not associated with increased risk of cancer
Proliferative without atypia
Characterized by accumulation of luminal epithelial cells
Small increased risk of cancer (1.5-2x general population)
Atypical hyperplasia
Change in size, shape, or nuclear function of epithelial cells
High risk of cancer development
Miscellaneous
Epidemiology
50% of women will experience a non-cancerous breast mass at some point in their lives
Age of diagnosis
Mean age of 51 years
Younger in proliferative
Older in atypical
Family History
Strongest in patients with atypical
NONPROLIFERATIVE DISORDERS
Breast Cysts
Most common
25% of nonproliferative
35-50 year olds
Fluid-filled, round mass originating from the terminal duct lobular unity
Patient Presentation
Painful or painless
Often solitary
Physical Examination
Smooth and firm to palpation with distinct border
Diagnostic Studies
Ultrasound
Simple
Anechoic throughout with posterior acoustic enhancement
Complicated
Homogenous low-level internal echoes with debris, thick walls, or thick septa
No solid components
Complex
Fluid and solid components without posterior wall enhancements
Management
Simple – no intervention required
Complicated – repeat imaging in 6 months
Complex – biopsy or FNA
Galactocele
Milk retention cysts usually caused by obstructed milk ducts
Physical Examination
Soft, cystic mass
Diagnostic Studies
Ultrasound
Complex echogenicity
Management
FNA reveals milky substance
No further intervention required
Hyperplasia of Usual Type
Increase in the number of epithelial cells within a duct that is more than two, but not more than four cells in depth and do not cross the lumen of the involved space
PROLIFERATIVE DISORDERS WITHOUT ATYPIA
Fibroadenoma
Most common benign tumor of the breast
50% of all breast biopsies
20% have multiple
Most common in younger women
15-35 years of age
Likely hormonally driven
Persist through reproductive year’s, increase during pregnancy or with estrogen therapies, and decrease after menopause
Physical Examination
Well-defined, mobile mass on palpation
Diagnostic Studies
Ultrasound
Well-defined solid mass with isoechogenicity
Management
Biopsy is indicated to further stratify
Simple
Contains glandular and fibrous tissue
Watch vs excision vs cryoablation
If any change during observation, then excision is warranted
Complex
Contains duct epithelial hyperplasia or calcification
Observation vs excision
Intraductal Papilloma
Papillary cells that grown from the wall of a cyst into its lumen
Can hide areas of atypia or ductal carcinoma in situ
Two types
Solitary
Solid mass on examination or incidental imaging
Nipple discharge is common presenting sign
Multiple
Minimum of five papillomas within a localized segment of tissue
Diagnostic Studies
Often found on routine mammography, but ultrasound is recommended if there is a palpable mass
Will show a mass within a cystic space
Management
Solitary
Biopsy and excision if atypical cells present
Multiple
Excision of breast segment
Sclerosing Adenosis
Lobular lesions with increased fibrous tissue and interspersed glandular cells
Found on routine mammography
Architectural distortion with irregular borders and microcalcifications
No interventions needed outside of routine imaging
Radial Scar
Complex sclerosing lesions found on routine imaging AFTER biopsies or excisions have been performed
Pathologic by definition due to appearance
Diagnostic Studies
Mammography often shows low-intensity, spiculated masses that are indistinguishable from spiculated carcinomas
Management
Biopsy reveals fibroelastic cores with radiating ducts and lobules
Excision is recommended (though controversial) and is often definitive
PROLIFERATIVE LESIONS WITH ATYPIA
Atypical Ductal Hyperplasia (ADH)
Characterized by proliferation of uniform epithelial cells with monomorphic round nuclei filling the involved duct
Must be < 2mm or involving < 2 ducts
Can share cytologic and morphologic features of low-grade ductal carcinoma in-situ
Diagnosed by core needle biopsy
Management
Excisional breast biopsy with good margins
Atypical Lobular Hyperplasia (ALH)
Characterized by proliferation of monomorphic, evenly spaced, dyshesive cells filling the involved lobule
Generally found on incidental biopsy for other clinical reason
Can share cytologic and morphologic features of low-grade lobular carcinoma in-situ
Diagnosed by core needle biopsy
Management
Excisional breast biopsy with good margins
Lobular Carcinoma in-situ
Invasive lesion that arises from the lobules and terminal ducts of the breast
80-90% of cases diagnosed in premenopausal women with a mean age of 45 year’s
Strong estrogen receptor positivity
Diagnosed by core needle biopsy on other incidental reason
LCIS is generally not diagnosed clinically, radiographically, or by gross pathologic examination
Three types
Classic
Solid proliferation of small cells with small, uniform round nuclei and variably distinct cell borders with cytologic dyshesion
Clear to lightly eosinophilic cytoplasm with possible signet ring cells and vacuoles
Pleomorphic
Larger cells with marked nuclear pleomorphism
Florid
Marked distension of the involved ducts and lobules that become mass-forming
Central necrosis with calcifications
Management
Excisional breast biopsy
Flat Epithelial Atypia
Characterized by neoplastic alteration of the terminal duct lobular units with replacement of the native epithelial cells with columnar cells
Diagnosed by core needle biopsy after mammographic evidence of microcalcifications
Management
Excisional breast biopsy
MISCELLANEOUS
Lipoma
Solitary mature fat tumors of the breast that do not contain histologic evidence of breast tissue
Physical Examination
Soft, non-tender, well-circumscribed mass
Difficulty to clinically differentiate from other conditions
Excisional biopsy is preferred
Fat Necrosis
Occurs as a result of breast trauma or surgical intervention
Can be confused with malignancy both clinically and radiographically
May see oil cysts on mammography or ultrasound
Biopsy is often needed to diagnose, but no further treatment is indicated
Diabetic Mastopathy
Seen in premenopausal women with long standing type 1 diabetes mellitus
Mammogram shows dense pattern
Diagnosed by core needle biopsy
Shows dense, keloid-like fibrosis with periductal, lobular, or perivascular lymphocytic infiltration
No further treatment after diagnosis
Hamartoma
Lesions containing varying amounts of glandular, adipose, or fibrous tissue
Present as discrete, encapsulated, painless masses found on incidental radiographic screening
FNA or CNB are not sufficient to make the diagnosis and excisional biopsy is preferred
Littrup PJ, Freeman-Gibb L, Andea A, et al. Cryotherapy for breast fibroadenomas. Radiology. 2005; 234(1):63-72. [pubmed]
Linda A, Zuiani C, Furlan A, et al. Radial scars without atypia diagnosed at imaging-guided needle biopsy: how often is associated malignancy found at subsequent surgical excision, and do mammography and sonography predict which lesions are malignant? AJR Am J Roentgenol. 2010; 194(4):1146-51. [pubmed]
27yo, G1P1001, presents to the OBGYN office with complaints of a “breast lump”. She states she first noticed it during her pregnancy last year, but did not think anything of it until her mother told her to have it checked it out. She denies any personal or family history of malignancies or any change in size since her pregnancy. Visual inspection shows no nipple retraction, skin dimpling, or asymmetry, and physical examination reveals a 1.5cm, well-defined, non-tender mobile mass 4cm from the nipple in the 1 o’clock position.
What would be the next step in the diagnostic evaluation of this lesion?
She is obviously concerned about breast cancer. Are they any concerning features in this presentation?
Other Known Aliases – artery of the round ligament of the uterus
Definition – branch of the inferior epigastric artery that runs under and supplies the round ligament of the uterus
Clinical Significance – this artery constitutes an anastomosis of the uterine and ovarian artery and is generally considered an physiologically insignificant artery dissected during hysterectomies. However, if accidentally severed or damaged, can lead to hemoperitoneum and need for re-operation.
History – Named after John Albertson Sampson (1873-1946), an American gynecologist who received his medical doctorate from Johns Hopkins University in 1899. He would spend the majority of his career at the Albany Hospital in New York and was a pioneer in the research of endometriosis, first introducing and coining the term for this condition in 1921. He would also be the first to describe the implantation areas of endometriosis as “chocolate cysts”. It was during his time at Johns Hopkins that he took a keen interest in oncology and extensively studied the lymphatic drainage and vascular supply of the pelvis, where he was later credited with his eponymous artery of the round ligament of the uterus.
References
Firkin BG and Whitwirth JA. Dictionary of Medical Eponyms. 2nd ed. New York, NY; Parthenon Publishing Group. 1996.
Bartolucci S, Forbis P. Stedman’s Medical Eponyms. 2nd ed. Baltimore, MD; LWW. 2005.
Yee AJ, Pfiffner P. (2012). Medical Eponyms (Version 1.4.2) [Mobile Application Software]. Retrieved http://itunes.apple.com.
Sampson J.A. Perforating hemorrhagic (chocolate) cysts of the ovary. Am J Obstet Gynecol. 1921;2:526–528. [Google Scholar]
Sampson J.A. Peritoneal endometriosis due to the menstrual dissemination of endometrial tissue into the peritoneal cavity. Am J Obstet Gynecol. 1927;14:422–469. [PMC free article] [PubMed] [Google Scholar]
Wong JW. Sampson’s Artery Revisited RMGO. 2017; 2:1-2. [link]
42yo G2P1001, who is 12-weeks gravid, presents to your office with concerns of public pain and a single episode of vaginal bleeding this morning. Physical examination reveals a gravid uterus with fundal height measured at the level of the umbilicus. Serum beta-hCG was found to be 745,223 mIU/mL. Ultrasound is below.
What are the important features of this case?
What is the condition that is present?
What is the next step in management?
Answer
The important features of this case are advanced maternal age (>35y), fundal height commensurate with a 20-week gestation, and a profoundly elevated beta hCG
2. The ultrasound video shows the classic “snow storm appearance” or “bunches of grapes” which is pathognomonic for hydatidiform mole, a type of gestational trophoblastic disease (GTD)
3. Initial management for GTD is methotrexate due to effectiveness, patient tolerance, and cost.
Definition – forced hyperflexion of the hips with applied suprapubic pressure during vaginal delivery
Clinical Significance – this is the primary maneuver to attempt to help relieve a shoulder dystocia during vaginal deliveries. Due to the hypermobilty of sacroilliac joint during pregnancy, this allows for rotation of the pelvis and facilitates releasing the stuck shoulder. It has been shown to have a success of close to 90%.
History – Named after William McRoberts, Jr. (1914-2006), an American obstetrician who recieved his medical doctorate from the University of Pittsburgh in 1940. He would go on to have a modest career in obstetrics culminating in Professor and Chief of Obstetrics at the University of Texas Medical School and Hermann Memorial Hospital in Dallas, TX. It was here where he his reputation as a teacher flourished and where he taught his eponymous maneuver for shoulder dystocia for over 40 years. As a testament to his teaching and a gift on retirement in 1982, two of his residents published an article naming this maneuver after their teacher and mentor.
References
Firkin BG and Whitwirth JA. Dictionary of Medical Eponyms. 2nd ed. New York, NY; Parthenon Publishing Group. 1996.
Bartolucci S, Forbis P. Stedman’s Medical Eponyms. 2nd ed. Baltimore, MD; LWW. 2005.
Yee AJ, Pfiffner P. (2012). Medical Eponyms (Version 1.4.2) [Mobile Application Software]. Retrieved http://itunes.apple.com.
