Tuesday, May 19, 2015

Does the PJS affect the kidneys?

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

(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, JG Park, M Miyaki, G Guanti, M C Costanza
Gut. 2006 July; 55(7): 984–990.  doi: 10.1136/gut.2005.082990
PMCID: PMC1856321

(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

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