A small apology

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.

Ep-PAINE-nym



Pemberton Sign

Other Known Aliases none

DefinitionRaising 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.

HistoryNamed 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

  1. Firkin BG and Whitwirth JA.  Dictionary of Medical Eponyms. 2nd ed.  New York, NY; Parthenon Publishing Group. 1996.
  2. Bartolucci S, Forbis P.  Stedman’s Medical Eponyms.  2nd ed.  Baltimore, MD; LWW.  2005.
  3. Yee AJ, Pfiffner P. (2012).  Medical Eponyms (Version 1.4.2) [Mobile Application Software].  Retrieved http://itunes.apple.com.
  4. Whonamedit – dictionary of medical eponyms. http://www.whonamedit.com
  5. Up To Date. www.uptodate.com
  6. Pemberton HS. SIGN OF SUBMERGED GOITRE The Lancet. 1946; 248(6423):509 [link]
  7. De Filippis EA, Sabet A, Sun MRM, Garber JR. Pemberton’s Sign: Explained Nearly 70 Years Later . 2014; 99(6):1949-1954 [link]

Ep-PAINE-nym



Heinz Bodies

Other Known Aliases Heinz-Ehrlich bodies, Ehrlich Inner Body

DefinitionDeep 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.

HistoryNamed 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

  1. Firkin BG and Whitwirth JA.  Dictionary of Medical Eponyms. 2nd ed.  New York, NY; Parthenon Publishing Group. 1996.
  2. Bartolucci S, Forbis P.  Stedman’s Medical Eponyms.  2nd ed.  Baltimore, MD; LWW.  2005.
  3. Yee AJ, Pfiffner P. (2012).  Medical Eponyms (Version 1.4.2) [Mobile Application Software].  Retrieved http://itunes.apple.com.
  4. Whonamedit – dictionary of medical eponyms. http://www.whonamedit.com
  5. Up To Date. www.uptodate.com
  6. 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

Ep-PAINE-nym



von Willebrand Disease

Other Known Aliases hereditary pseudohemophilia

DefinitionAutosomal 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.

HistoryNamed 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

  1. Firkin BG and Whitwirth JA.  Dictionary of Medical Eponyms. 2nd ed.  New York, NY; Parthenon Publishing Group. 1996.
  2. Bartolucci S, Forbis P.  Stedman’s Medical Eponyms.  2nd ed.  Baltimore, MD; LWW.  2005.
  3. Yee AJ, Pfiffner P. (2012).  Medical Eponyms (Version 1.4.2) [Mobile Application Software].  Retrieved http://itunes.apple.com.
  4. Whonamedit – dictionary of medical eponyms. http://www.whonamedit.com
  5. Up To Date. www.uptodate.com
  6. Von Willebrand EA. Hereditary pseudohaemophilia. Haemophilia. 1999; 5(3):223-31. [translation of original paper] [pubmed]
  7. Leebeek FW, Eikenboom JC. Von Willebrand’s Disease. NEJM. 2016; 375(21):2067-2080. [pubmed]
  8. Nilsson IM. Commentary to Erik von Willebrand’s original paper from 1926 ‘Hereditär pseudohemofili’. Haemophilia. 1999; 5(3):220-1. [pubmed]

PAINE #PANCE Pearl – Gynecology



Question

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.

  1. What would be the next step in the diagnostic evaluation of this lesion?
  2. She is obviously concerned about breast cancer. Are they any concerning features in this presentation?

Answer

  1. 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.
  2. 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.

#69 – Benign Breast Diseases



***LISTEN TO THE PODCAST HERE***



Anatomy and Physiology

  • 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

1893 Cottage Physician


References

  1. https://armandoh.org/disease/breast-cancer/
  2. Schnitt SJ. Benign breast disease and breast cancer risk: morphology and beyond. Am J Surg Pathol. 2003; 27(6):836-41. [pubmed]
  3. Hartmann LC, Sellers TA, Frost MH, et al. Benign breast disease and the risk of breast cancer. N Engl J Med. 2005; 353(3):229-37. [pubmed]
  4. Guray M, Sahin AA. Benign breast diseases: classification, diagnosis, and management. Oncologist. 2006; 11(5):435-49. [pubmed]
  5. Breast Disease. In: Hoffman BL, Schorge JO, Halvorson LM, Hamid CA, Corton MM, Schaffer JI. eds. Williams Gynecology, 4e. McGraw-Hill; Accessed February 21, 2021. https://accessmedicine-mhmedical-com.ezproxy.uthsc.edu/content.aspx?bookid=2658&sectionid=218608871
  6. Giuliano AE, Hurvitz SA. Breast Disorders. In: Doherty GM. eds. Current Diagnosis & Treatment: Surgery, 15e. McGraw-Hill; Accessed February 21, 2021. https://accessmedicine-mhmedical-com.ezproxy.uthsc.edu/content.aspx?bookid=2859&sectionid=242155824
  7. Littrup PJ, Freeman-Gibb L, Andea A, et al. Cryotherapy for breast fibroadenomas. Radiology. 2005; 234(1):63-72. [pubmed]
  8. 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]
  9. American Society of Breast Surgeons. Official statement. Consensus guideline on concordance assessment of image-guided breast biopsies and management of borderline or high-risk lesions. 2016. Available at: https://www.breastsurgeons.org/docs/statements/Consensus-Guideline-on-Concordance-Assessment-of-Image-Guided-Breast-Biopsies.pdf
  10. Guray M, Sahin AA. Benign breast diseases: classification, diagnosis, and management. Oncologist. 2006; 11(5):435-49. [pubmed]

PAINE #PANCE Pearl – Gynecology



Question

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.

  1. What would be the next step in the diagnostic evaluation of this lesion?
  2. She is obviously concerned about breast cancer. Are they any concerning features in this presentation?

Ep-PAINE-nym



Sampson’s Artery

Other Known Aliases artery of the round ligament of the uterus

Definitionbranch 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.

HistoryNamed 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

  1. Firkin BG and Whitwirth JA.  Dictionary of Medical Eponyms. 2nd ed.  New York, NY; Parthenon Publishing Group. 1996.
  2. Bartolucci S, Forbis P.  Stedman’s Medical Eponyms.  2nd ed.  Baltimore, MD; LWW.  2005.
  3. Yee AJ, Pfiffner P. (2012).  Medical Eponyms (Version 1.4.2) [Mobile Application Software].  Retrieved http://itunes.apple.com.
  4. Whonamedit – dictionary of medical eponyms. http://www.whonamedit.com
  5. Up To Date. www.uptodate.com
  6. Sampson J.A. Perforating hemorrhagic (chocolate) cysts of the ovary. Am J Obstet Gynecol. 1921;2:526–528. [Google Scholar]
  7. 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]
  8. Wong JW. Sampson’s Artery Revisited RMGO. 2017; 2:1-2. [link]

PAINE #PANCE Pearl – Obstetrics



Question

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.

  1. What are the important features of this case?
  2. What is the condition that is present?
  3. What is the next step in management?

Answer

  1. 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.

Ep-PAINE-nym



McRoberts Manuever

Other Known Aliases none

Definitionforced 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%.

HistoryNamed 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

  1. Firkin BG and Whitwirth JA.  Dictionary of Medical Eponyms. 2nd ed.  New York, NY; Parthenon Publishing Group. 1996.
  2. Bartolucci S, Forbis P.  Stedman’s Medical Eponyms.  2nd ed.  Baltimore, MD; LWW.  2005.
  3. Yee AJ, Pfiffner P. (2012).  Medical Eponyms (Version 1.4.2) [Mobile Application Software].  Retrieved http://itunes.apple.com.
  4. Whonamedit – dictionary of medical eponyms. http://www.whonamedit.com
  5. Up To Date. www.uptodate.com
  6. Gonik B, Stringer CA, Held B. An alternate maneuver for management of shoulder dystocia. Am J Obstet Gynecol. 1983; 145(7):882-4. [pubmed]