Male Genital Tract Part 2

Front 1

What is shown here?

What percent of boys? What age commonly?

What percent unilateral/bilateral?

What is the pathogenesis?

What are the two phases of development and what occurs in each? What phase does this defect most commonly occur in?

Back 1

Front 2

Back 2

Front 3

Which is normal, which is cryptorchid with total absence of spermatogenesis?

What are two complications of cryptorchidism?

Decreased spermatogenesis in ______ testes in unilateral cryptorchidism.

Systemic mechanism, poorly understood (not just “______testis”)
Orchiopexy before age ______ improves (but does not guarantee) chances of normal spermatogenesis
Neoplasms
______fold increase in risk of malignant neoplasm in cryptorchid testis
Some increased risk in ______ normally descended testis. Both risks are ______ but not totally eliminated by orchiopexy

Back 3

Inguinal testis is susceptible to trauma

Sterility

Decreased spermatogenesis in BOTH testes in unilateral cryptorchidism
Systemic mechanism, poorly understood (not just “overheated testis”)
Orchiopexy before age 2 improves (but does not guarantee) chances of normal spermatogenesis
Neoplasms
5-10 fold increase in risk of malignant neoplasm in cryptorchid testis
Some increased risk in contra lateral normally descended testis
Both risks are reduced but not totally eliminated by orchiopexy

Front 4

What are some physical signs of Klinefelter Syndrome?

What genotype do 80% have? Are the gonads functional?

What is the frequency?

Back 4

Front 5

•Klinefelter Syndrome:

Clinical:
◦______ appearance with increased stature and small to ______l-sized, well developed testes.
◦Incomplete ______.
◦Gynecomastia.
◦Mental retardation, ______ difficulties.
•Histology:
◦Small hyalinized _______ tubules.
◦Pseudoadenomatous clusters of ______ cells – these cells only appear to be increased in number, though, because the decreased ______ volume.
•Associations: increased incidence of extragonadal ______ cell tumors (mediastinum>______, CNS, retroperitoneum) as well as hypopituitarism.

Back 5

•Clinical:
◦Eunuchoid appearance with increased stature and small to normal-sized, well developed testes.
◦Incomplete virilization.
◦Gynecomastia.
◦Mental retardation, speech difficulties.
•Histology:
◦Small hyalinized seminiferous tubules.
◦Pseudoadenomatous clusters of Leydig cells – these cells only appear to be increased in number, though, because the decreased testicular volume.
•Associations: increased incidence of extragonadal germ cell tumors (mediastinum>pineal gland, CNS, retroperitoneum) as well as hypopituitarism.

Front 6

Normal on right

This testicular biopsy is from an adult male with history of normal semen volume and severe oligospermia. He had small firm testes and body habitus suggestive of Klinefelter’s syndrome. The biopsy shows small hyalinized seminiferous tubules and pseudo-adenomatous clusters of Leydig cells.
Contrast with normal testis on the right.

What is the condition?

Back 6

Klinefelter syndrome

Front 7

How would you describe the testicular tubules here? What is the most common infectious cause for this finding?

Back 7

Focal atrophy

Mumps orchitis

Front 8

Mumps infection post puberty may be complicated by ______ in a fourth to a ______ of cases. In general, the orchitis is ______ (bilateral or unilateral) and patchy so that sterility following ______ is uncommon. Other infectious agents associated with orchitis may include echovirus, lymphocytic ______ virus, influenza virus, ______virus, and arboviruses. In contrast, ______ is a more frequent cause for scrotal pain and swelling in adult males and is most likely to be the result of a ______ disease such as Chlamydia trachomatis or ______ in younger males or gram negative bacteria from urinary tract infection of ______ males. Disseminated tuberculosis may occasionally involve the ______.

Back 8

Mumps infection post puberty may be complicated by orchitis in a fourth to a third of cases. In general, the orchitis is unilateral and patchy so that sterility following infection is uncommon. Other infectious agents associated with orchitis may include echovirus, lymphocytic choriomeningitis virus, influenza virus, Coxsackie virus, and arboviruses. In contrast, epididymitis is a more frequent cause for scrotal pain and swelling in adult males and is most likely to be the result of a sexually transmissible disease such as Chlamydia trachomatis or Neisseria gonorrheae in younger males or gram negative bacteria from urinary tract infection of older males. Disseminated tuberculosis may occasionally involve the epididymis.

Front 9

What is shown here? Which phase? What is the pathway of infection of this phase. Contrast this with gonorrhea.

Back 9

Tertiary syphilis (spirochetes)

Testis => then epididymis

Gonorrhea => retrograde from urethra to the prostate => seminal vesicles => epididymis => prostate

Front 10

What is the disease shown in the testes here? How does it spread?

Back 10

Typically, tuberculosis spreads retrograde from the prostate to the epididymis, then to the testis. The left panel shows involvement of both the epididymis and the testis; microscopically, a granuloma is seen in the epididymis. It is a very young granuloma, so typical caseation is not yet seen.

Front 11

What is this?

Back 11

Testis = granulomatous orchitis

Front 12

Idiopathic granulomatous orchitis is an uncommon ______ testicular lesion that follows a gram ______ urinary tract infection in the majority of cases. It is most prevalent in the ___ and ___ decades. Testicular involvement is usually diffuse but may also present as a localized ______ lesion and may simulate a testicular ______. The epididymis and ______ may also be involved.
The inflammatory process is predominantly intratubular with the cellular infiltrate containing a majority of histiocytes admixed with ______ and plasma cells. Giant cells may also be present. The predominance of ______ imparts a granulomatous appearance but distinct ______ are not formed. Non-specific chronic interstitial inflammation is also a feature of this condition.
The ______ localization of inflammation in granulomatous orchitis aids in its distinction from infectious ______ (mycobacterial, leprosy, brucellosis etc.) and sarcoidosis which are predominantly ______ processes. ______ is not seen in granulomatous orchitis.

A related process is autoimmune ______, which is clinically described as rapid onset testicular enlargement in ______ aged men which may be associated with a febrile illness. Histology shows ______ without organisms. An autoimmune etiology is proposed.

Back 12

Idiopathic granulomatous orchitis is an uncommon inflammatory testicular lesion that follows a gram negative urinary tract infection in the majority of cases. It is most prevalent in the 5th and 6th decades. Testicular involvement is usually diffuse but may also present as a localized nodular lesion and may simulate a testicular neoplasm. The epididymis and spermatic cord may also be involved.
The inflammatory process is predominantly intratubular with the cellular infiltrate containing a majority of histiocytes admixed with lymphocytes and plasma cells. Giant cells may also be present. The predominance of histiocytes imparts a granulomatous appearance but distinct granulomas are not formed. Non-specific chronic interstitial inflammation is also a feature of this condition.
The intratubular localization of inflammation in granulomatous orchitis aids in its distinction from infectious granulomas (mycobacterial, leprosy, brucellosis etc.) and sarcoidosis which are predominantly interstitial processes. Necrosis is not seen in granulomatous orchitis.

A related process is autoimmune orchitis, which is clinically described as rapid onset testicular enlargement in middle aged men which may be associated with a febrile illness. Histology shows granulomas without organisms. An autoimmune etiology is proposed.

Front 13

What is the mnemonic for testicular regression?

Back 13

V—Vascular conditions bring to mind varicoceles, which cause atrophy on the side of the dilated veins.
I—Inflammation recalls the atrophy following mumps orchitis and other causes of epidydimoorchitis.
N—Neoplasms suggest the atrophy that occurs in the estrogen treatment of prostatic carcinoma.
D—Degenerative suggests the atrophy resulting from aging.
I—Intoxication should remind one of the atrophy resulting from chronic alcoholism, Laennec cirrhosis, and hemochromatosis. X-ray exposure may also produce atrophy.
C—Congenital recalls undescended testes and torsion.
A—Autoimmune and allergic suggest nothing.
T—Trauma reminds one of the atrophy following vasectomy and accidental ligation of the blood supply during hernia repair.
E—Endocrine suggests the atrophy of hypopituitarism, Klinefelter syndrome, and other eunuchoidal states

Front 14

What is this an image of?

Back 14

Torsion of the testis

Front 15

Torsion is twisting of the ______, leading to ischemia and ______ stasis. It may be related to trauma, but frequently the inciting event is ______. Often there will be a predisposing anatomic abnormality which allows the testis excess ______ within the scrotum (“bell clapper phenomenon”); this abnormality may be ______, which is why there is a risk of contra lateral torsion in a patient who has torsion. This is a true urologic emergency, since surgery within ______ hours may save the testis; after that, hemorrhagic ______ with obliteration of the testis is inevitable if reduction of the torsion is too late.

Back 15

Torsion is twisting of the spermatic cord, leading to ischemia and venous stasis. It may be related to trauma, but frequently the inciting event is obscure. Often there will be a predisposing anatomic abnormality which allows the testis excess mobility within the scrotum (“bell clapper phenomenon”); this abnormality may be bilateral, which is why there is a risk of contra lateral torsion in a patient who has torsion. This is a true urologic emergency, since surgery within 4-6 hours may save the testis; after that, hemorrhagic infarction with obliteration of the testis is inevitable if reduction of the torsion is too late.

Front 16

What is the condition (what would cause the seminiferous tubules to become necrotic)?

Back 16

Torsion of the testis

Front 17

What is shown in the image? What are 50% of germ cell tumors? What is the histologic hallmark?

What are 15% of germ cell tumors produce?

Back 17

Germ cell tumors:
Seminomas (50% of germ cell tumors)
Most common germ cell tumor to occur in “pure” form
Grossly homogeneous, not typically necrotic or hemorrhagic
Microscopically has characteristic distinct cell membrane and clear cytoplasm giving a “fried egg” appearance to the cells
15% produce human chorionic gonadotropin (HCG)

Front 18

Embryonal carcinoma (occasionally a ______ tumor, but much more commonly a component of a mixed ______ cell tumor)
______ aggressive than seminomas
Grossly more likely to be ______ or necrotic
Microscopically shows sheets, ______, tubules or papillary formations—generally ______ looking cells

Back 18

Embryonal carcinoma (occasionally a pure tumor, but much more commonly a component of a mixed germ cell tumor)
More aggressive than seminomas
Grossly more likely to be hemorrhagic or necrotic
Microscopically shows sheets, alveoli, tubules or papillary formations—generally anaplastic looking cells

Front 19

What type of tumor? (______ sinus tumor)
Almost exclusively a tumor of ______ and children up to ___ years of age
In adults, more commonly seen mixed with other ______ cell tumor patterns
Main distinguishing feature is production of ______ which serves as a serum marker
Immunohistochemical stain (lower right) is positive for______ = SHILLER-DUVALL BODIES?

Back 19

Yolk sac tumor (endodermal sinus tumor)
Almost exclusively a tumor of infants and children up to 3 years of age
In adults, more commonly seen mixed with other germ cell tumor patterns
Main distinguishing feature is production of alpha fetoprotein (AFP) which serves as a serum marker
Immunohistochemical stain (lower right) is positive for alpha fetoprotein = SHILLER-DUVALL BODIES?

Front 20

What is alpha-fetal protein?

Back 20

Fetal albumin

Front 21

What is the tumor type?

Only ___% of germ cell tumors are pure chorio, but chorio is commonly represented in mixed ______ cell tumors.
Highly aggressive
Mimics the histology of normal ______ (syncitiotropoblasts and cytotrophoblasts) and produces ______ (used as a tumor marker)
Grossly and microscopically a very ______ tumor…metastases bleed easily.
A s________ type cell staining positively for HCG is seen in the lower right panel.

Back 21

Only 1% of germ cell tumors are pure chorio, but chorio is commonly represented in mixed germ cell tumors.
Highly aggressive
Mimics the histology of normal placenta (syncitiotropoblasts and cytotrophoblasts) and produces HCG (used as a tumor marker)
Grossly and microscopically a very vascular tumor…metastases bleed easily
A syncitiotrophoblast type cell staining positively for HCG is seen in the lower right panel.

Front 22

What type of tumor is this? (that's cartilage...)

Back 22

Teratoma!

Front 23

Teratoma:

In pure form, teratoma is more common in ______…rare in ______; it is commonly mixed with other ______ cell tumor types in adults

Mature types (with ______ tissues) and immature types with (______ tissues) exist

Tissue represents more than one _______ layer. May be grossly quite large with variegated color and texture; ______ are common

This photo represents a mature testicular teratoma in a 22 year old; mature cartilage and skin are represented in the photomicrographs.

Back 23

Teratoma
In pure form, teratoma is more common in children…rare in adults; it is commonly mixed with other germ cell tumor types in adults
Mature types (with adult tissues) and immature types with (fetal tissues) exist
Tissue represents more than one germ layer
May be grossly quite large with variegated color and texture; cysts are common
This photo represents a mature testicular teratoma in a 22 year old; mature cartilage and skin are represented in the photomicrographs.

Front 24

What is the tumor type? What type of tissue does it demonstrate?

Back 24

Germ cell tumors:
Teratoma
This immature teratoma is not grossly different from the previous example; microscopically, though, it demonstrates immature neuroectodermal tissue (upper right) and fetal type stromal tissue (lower right)

Front 25

Remind your male patients to do testicular self exams just as you remind your female patients to do breast self exams; you very well may save a life!

Back 25

Remind your male patients to do testicular self exams just as you remind your female patients to do breast self exams; you very well may save a life!