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Sex-Cord Tumor with Annular Tubules with Unusual Morphology in an Infant with Peutz-Jeghers Syndrome.
Fetal Pediatr Pathol. 2020 Jul 01 [Online ahead of print]FP

Abstract

Background:

Peutz-Jeghers syndrome (PJS) is characterized by hamartomatous gastrointestinal polyposis, mucocutaneous pigmentation and cancer predisposition. The clinical features of PJS manifest in first two decades of life; however, neonatal presentation is uncommon. Case report: We present a five day old girl with PJS that presented with obstructive hamartomatous polyps in the sigmoid colon. At colostomy closure at six months, an incidental ovarian sex-cord tumor with annular tubules (SCTAT) was detected. It showed predominantly a solid pattern with limited tubule formation and was composed of lipid-rich cells. She had no hormonal symptoms.

Conclusion:

SCTAT can occur as young as six months of age in PJS, and may show histologic overlap with lipid-rich Sertoli cell tumors.

Authors+Show Affiliations

Department of Pathology, Institute of Post Graduate Medical Education & Research, Kolkata, India.Department of Pathology, Medical College and Hospital Kolkata, India.Department of Pathology, Institute of Post Graduate Medical Education & Research, Kolkata, India.Department of Pediatric Surgery, Park Clinic, Kolkata, India.

Pub Type(s)

Journal Article

Language

eng

PubMed ID

32609034

Citation

Maity, Priyanka, et al. "Sex-Cord Tumor With Annular Tubules With Unusual Morphology in an Infant With Peutz-Jeghers Syndrome." Fetal and Pediatric Pathology, 2020, pp. 1-6.
Maity P, Das N, Chatterjee U, et al. Sex-Cord Tumor with Annular Tubules with Unusual Morphology in an Infant with Peutz-Jeghers Syndrome. Fetal Pediatr Pathol. 2020.
Maity, P., Das, N., Chatterjee, U., & Basak, D. (2020). Sex-Cord Tumor with Annular Tubules with Unusual Morphology in an Infant with Peutz-Jeghers Syndrome. Fetal and Pediatric Pathology, 1-6. https://doi.org/10.1080/15513815.2020.1786200
Maity P, et al. Sex-Cord Tumor With Annular Tubules With Unusual Morphology in an Infant With Peutz-Jeghers Syndrome. Fetal Pediatr Pathol. 2020 Jul 1;1-6. PubMed PMID: 32609034.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Sex-Cord Tumor with Annular Tubules with Unusual Morphology in an Infant with Peutz-Jeghers Syndrome. AU - Maity,Priyanka, AU - Das,Nandini, AU - Chatterjee,Uttara, AU - Basak,Dhananjay, Y1 - 2020/07/01/ PY - 2020/7/2/entrez KW - Peutz-Jeghers syndrome KW - Sertoli cell tumors KW - sex-cord tumor with annular tubules KW - sex-cord tumors SP - 1 EP - 6 JF - Fetal and pediatric pathology JO - Fetal Pediatr Pathol N2 - Background: Peutz-Jeghers syndrome (PJS) is characterized by hamartomatous gastrointestinal polyposis, mucocutaneous pigmentation and cancer predisposition. The clinical features of PJS manifest in first two decades of life; however, neonatal presentation is uncommon. Case report: We present a five day old girl with PJS that presented with obstructive hamartomatous polyps in the sigmoid colon. At colostomy closure at six months, an incidental ovarian sex-cord tumor with annular tubules (SCTAT) was detected. It showed predominantly a solid pattern with limited tubule formation and was composed of lipid-rich cells. She had no hormonal symptoms. Conclusion: SCTAT can occur as young as six months of age in PJS, and may show histologic overlap with lipid-rich Sertoli cell tumors. SN - 1551-3823 UR - https://www.unboundmedicine.com/medline/citation/32609034/Sex-Cord_Tumor_with_Annular_Tubules_with_Unusual_Morphology_in_an_Infant_with_Peutz-Jeghers_Syndrome L2 - http://www.tandfonline.com/doi/full/10.1080/15513815.2020.1786200 DB - PRIME DP - Unbound Medicine ER -
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