Sex cord stromal tumors

Sex cord tumor with annular tubules

Last author update: 1 December 2012
Last staff update: 25 May 2023 (update in progress)

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PubMed search: Ovarian sex cord tumor with annular tubules

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Cite 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,
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


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


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
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