Table of Contents
Definition / general | Gross images | Microscopic (histologic) description | Microscopic (histologic) images | Electron microscopy description | Differential diagnosisCite this page: Ehdaivand S. Sex cord tumor with annular tubules. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/ovarytumorsexcordannular.html. Accessed June 10th, 2023.
Definition / general
- Distinctive ovarian tumor associated with hyperestrinism (50%)
- 1/3 with tumor have Peutz-Jegher syndrome
- Peutz-Jegher syndrome: autosomal dominant with variable penetrance; patients have ovarian sex cord stromal tumors or tumorlets with annular tubules (almost all patients, usually bilateral, often with calcifications, tumorlets are benign); mucocutaneous melanin pigmentation (lips, oral mucosa, digits, palms, soles, genitalia); GI hamartomatous polyposis (florid epithelium supported by broad bands of smooth muscle in stalk, causes intussusception and GI bleeding); occasional adenocarcinomas of GI tract, pancreas, breast, lung; occasional cervical adenoma malignum, ovarian mucinous carcinomas; Peutz was Dutch physician 1886-1957; Jeghers was American physician born 1904
- Patients without Peutz-Jegher syndrome have large, unilateral tumors, with transitions to granulosa cell tumors, 40% secrete estrogen and 20% are malignant
- Arise in ovarian cortex from follicular granulosa cells
Gross images
AFIP images
Large, yellow-orange,
unilateral,
predominantly
solid tumor
Microscopic (histologic) description
- Mixture of simple and complex annular tubules with eosinophilic hyaline bodies, often calcified
- Resembles granulosa cell tumor with Sertoli growth pattern
- Simple annular tubules are ring shaped, with peripheral oriented nuclei around a central hyalinized body composed of basement membrane material
- Most of ring is anuclear cytoplasmic zone
- Complex annular tubules are made of intercommunicating rings revolving around multiple hyaline bodies, often calcified
Microscopic (histologic) images
AFIP images
Simple and complex annular tubules
Scattered annular tubules
Islands of vacuolated sex cord cells loaded with lipid
Diffuse growth of cells
Calcification
Extensive hyalinization in tubules and stroma
Area of microfollicular granulosa cell tumor
Area of Sertoli cell tumor
10 year old girl with precocious puberty:
Various images
Alpha-inhibin
Contributed by Sleiman Khalil, M.D.
22 year old Somali woman with 17 cm ovarian tumor
Electron microscopy description
- Granulosa cell and Sertoli cell features (granulosa cell-deeply indented nuclei, interdigitating plasma membranes joined by abundant desmosomes; Sertoli cell-variable Charcot-Bottcher filaments as cytoplasmic inclusions, Am J Clin Pathol 1981;75:11)
Differential diagnosis
- Gonadoblastoma: different clinical background, has germ cell component
- Granulosa cell tumor
- Sertoli cell tumor
