Just the sort of odd
question I like. And one I've lived with it for years since hearing of a 30
y.o. man with PJS and kidney mass (probably a cyst, though I never learned the
final pathology report).
A 60 y.o. woman who died of
stomach carcinoma had a history of bladder papillomas. (1)
A 27 y.o. man had multiple
urinary tract polyps affecting his ureter (the tube that connects kidney &
bladder). (2)
There was also a report of a
family with both PJS and polycystic kidneys, though I couldn't tell from the
title whether the two conditions were linked or coexisted. (3)
And a 4 y.o. girl with a
bladder hamartoma. (4)
Two patients with kidney
(renal) cancer reported from France. (5)
Another patient with PJS
& kidney cancer is reported by Linos of Mayo Clinic in 1981. The abstract
of this very odd article concludes, "During the 45-year period between
1935 and 1979, 48 patients with Peutz-Jeghers syndrome were
seen at the Mayo Clinic,
Rochester, Minn, and followed up for a median period of 33 years. Carcinoma of
the small or large intestine did not develop in any patient, with possibly one
exception." While the article itself contains the following contradictory
information, "During this 45 year period, carcinomas developed in six of
the 21 patients as follow: lung, two patients; breast, one patient; biliary
tree, one patient; kidney, one patient; and uterus, one patient. One patient
with a previous history of breast cancer and bilateral ovarian cystadenomas was
found to have abdominal carcinomatosis. Pathological examination showed
metastatic grade 1 mucous adenocarcinoma, but it was not possible to determine
whether the primary lesion was of colonic or ovarian origin." (6)
Here's report of a PJS woman
in Spain with three types of cancer including a right kidney carcinoma, rectal
carcinoma and cholangiocarcinoma. (7)
One PJS patient with kidney
cancer. (8)
A female PJS patient with
three different primary cancers (breast, thyroid and kidney). (9)
If you know of
urinary tract tumors in PJS people, please let me know - pj4steph@aol.com
References:
(1) Gastrointestinal Polyposis with
Mucocutaneous Pigmentation
Dormandy, T.L.,:
New Eng J Med
256:1093-1103;1141-1146;1186-1190 (June 6) 1957.
FULL TEXT IS AVAILABLE FREE!
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1889517/
(2) Peutz-Jeghers syndrome
and ureteral polyposis.
Sommerhaug RG, Mason T.
JAMA. 1970 Jan
5;211(1):120-2. No abstract available.
PMID: 5466889 [PubMed -
indexed for MEDLINE]
(3) Mucocutaneous
pigmentation and intestinal polyposis (Peutz-Jeghers syndrome) in a family of
Iraqi jews with polycystic kidney disease. With a chromosome study.
Kieselstein M, Herman G,
Wahrman J, Voss R, Gitelson S, Feuchtwanger M, Kadar S.
Isr J Med Sci. 1969
Jan-Feb;5(1):81-90. No abstract available.
PMID: 5796283 [PubMed - indexed
for MEDLINE]
(4) Hamartoma of the bladder
in a 4-year-old girl with hamartomatous polyps of the gastrointestinal tract.
Keating MA, Young RH,
Lillehei CW, Retik AB.
J Urol. 1987
Aug;138(2):366-9.
PMID: 3599256
(5) Peutz-Jeghers families
unlinked to STK11/LKB1 gene mutations are highly predisposed to primitive
biliary adenocarcinoma.
Olschwang S, Boisson C,
Thomas G.
J Med Genet. 2001
Jun;38(6):356-60.
PMID: 11389158 [PubMed -
indexed for MEDLINE]
FULL TEXT IS AVAILABLE FREE!
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1734902/
(6) Does Peutz-Jeghers
syndrome predispose to gastrointestinal malignancy? A later look.
Linos DA, Dozois RR, Dahlin
DC, Bartholomew LG.
Arch Surg. 1981
Sep;116(9):1182-4.
PMID: 7283716 [PubMed -
indexed for MEDLINE]
(7) [Peutz-Jeghers syndrome
associated with multiple epithelial tumors]
Cosme A, Ojeda E, San
Vicente MT, Barrio J, Bujanda L, Lopez P.
Servicio de Digestivo,
Hospital Donostia, Edificio Aranzazu, San Sebastian.
Gastroenterol Hepatol. 2001
Dec;24(10):495-9.
PMID: 11730618
(8) Cancer risks in LKB1
germline mutation carriers
H Mehenni, N Resta, J‐G Park, M
Miyaki, G Guanti, M C Costanza
Gut. 2006 July; 55(7):
984–990. doi: 10.1136/gut.2005.082990
PMCID: PMC1856321
FULL TEXT IS AVAILABLE FREE!
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1856321/?report=reader
(9) Findings from the
Peutz-Jeghers Syndrome Registry of Uruguay
Asadur Tchekmedyian,
Christopher I. Amos, Sherri J. Bale, Dakai Zhu, Stefan Arold, Joaquin Berrueta,
Natalie Nabon, Thomas McGarrity
PLoS One. 2013; 8(11):
e79639. Published online 2013 November
19. doi: 10.1371/journal.pone.0079639
PMCID: PMC3834183
FULL TEXT IS AVAILABLE FREE!
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