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| LETTER TO EDITOR |
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| Year : 2021 | Volume
: 14
| Issue : 1 | Page : 166-167 |
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Incidental finding of non-Peutz-Jeghers syndrome-associated ovarian sex cord tumor with annular tubules
Kavita Mardi
Department of Pathology, Indira Gandhi Medical College, Shimla, Himachal Pradesh, India
| Date of Submission | 26-Aug-2020 |
| Date of Acceptance | 15-Oct-2020 |
| Date of Web Publication | 09-Feb-2021 |
Correspondence Address:
Dr. Kavita Mardi
12-A, Type V Quarters, GAD Colony, Kasumpti, Shimla, Himachal Pradesh
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/kleuhsj.kleuhsj_272_20
How to cite this article:
Mardi K. Incidental finding of non-Peutz-Jeghers syndrome-associated ovarian sex cord tumor with annular tubules. Indian J Health Sci Biomed Res 2021;14:166-7 |
How to cite this URL:
Mardi K. Incidental finding of non-Peutz-Jeghers syndrome-associated ovarian sex cord tumor with annular tubules. Indian J Health Sci Biomed Res [serial online] 2021 [cited 2021 Feb 25];14:166-7. Available from: https://www.ijournalhs.org/text.asp?2021/14/1/166/308961 |
Dear Editor,
We would like to report an incidental finding of microscopic foci of ovarian sex cord tumor with annular tubules (SCTAT) in a patient with unsuspected Peutz–Jeghers syndrome (PJS).
Total abdominal hysterectomy with bilateral salphingo-oophorectomy was carried out in a 37-year-old patient who presented with menorrhagia of 2 years' duration. Gross examination revealed multiple intramural fibroids. Both ovaries were grossly within normal limits. However, microscopic examination of the right ovary revealed multiple foci of aggregates of distinctive tubular structures in the cortical region of the right ovary [Figure 1]. Each tubular structure consisted of cells with basally situated rows of bland nuclei and abundant supranuclear, clear-to-vacuolated cytoplasm that were concentrically arranged around the hyaline or eosinophilic basement membrane-like material [Figure 2]. In many tubules, the nuclei were arranged immediately subjacent to the basement membrane-like material. Simple ring-like tubular forms, as well as complex cribriform-type structures, were present. With these microscopic foci of SCTAT in the right ovary, the patient was investigated further for PJS. However, the patient neither had mucocutaneous pigmentation nor showed any evidence of hamartomatous polyps in the intestine. | Figure 1: Microphotograph showing foci of aggregate of distinctive tubular structures surrounded by the ovarian stroma (H and E, ×20)
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 | Figure 2: Higher magnification showing cells with basally situated rows of bland nuclei and abundant supranuclear, clear-to-vacuolated cytoplasm that were concentrically arranged around the hyaline or eosinophilic basement membrane-like material (H and E, ×40)
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The histogenesis and/or direction of differentiation of SCTAT has been a source of some disagreement in the literature. As noted previously, SCTAT has morphologic features intermediate between granulosa cells and Sertoli cell tumors. Other authors, primarily based on ultrastructural findings, have favored either a granulosa cell[1] or a Sertoli cell origin.
Because SCTAT occurring in patients with PJS is of typically microscopic, often incidental findings, the question arises as to the true nature of these lesions, that is, whether they are truly neoplasms or merely represent hyperplastic nodules of granulosa cells or hamartomas. Delides et al.[2] carefully sectioned 588 ovaries reported to be of normal size and identified characteristic SCTAT in seven cases, with an average diameter of 0.17 mm. The authors suggested to use the term “nest” instead of “tumor” for these small SCTAT lesions in ovary to reflect their true nature. In their seminal report, Scully et al.[3] rationalized the use of the term “tumor” by referring to the spectrum of sizes in these lesions: “The fact that lesions of all sizes up to large tumor have been observed supports the concept that all of them are neoplastic, varying only in size.”
With respect to the biologic behavior and malignant potential, cases of SCTAT occurring in PJS patients also appear to be different from that of their non-PJS counterparts. In the former group, all the reported cases with one exception have behaved in a benign fashion.[4] Tumors in patients without evidence of PJS are unilateral and are usually large. They are usually accompanied by symptoms suggestive of hyperestrinism, such as menstrual irregularity, postmenopausal bleeding, or sexual precocity. The incidence of malignancy and mortality is also higher in these patients.[3] In further support of the dichotomy between the PJS-associated SCTAT and the non-PJS-associated cases are the molecular findings recently reported by Connolly et al.[5] Mutations in the PJS-associated gene STK11, which maps to 19p13.3, were recently found in two PJS-associated SCTAT cases and in none of the five non-PJS-associated SCTAT cases.[5]
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the legal guardian has given his consent for images and other clinical information to be reported in the journal. The guardian understands that names and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
| References | |  |
| 1. | Crissman JD, Hart WR. Ovarian sex cord tumors with annular tubules: an ultrastructural study of three cases. Am J Clin Pathol 1981;75:11-17.  |
| 2. | Delides GS, Elemenoglou J, Garas G. Sex cord nests with annular tubules: the incidence of identifying them in normal ovaries. Acta Morphol Hung 1988;36:3-6. |
| 3. | Young RH, Welch WR, Dickerson GR, Scully RE. Ovarian sex cord tumor with annular tubules. Review of 74 cases including 27 with Peutz-Jeghers syndrome and four with adenoma malignum of the cervix. Cancer 2006;50:1384-402. |
| 4. | Lele SM, Sawh RN, Zaharopoulos P. Malignant ovarian sex cord tumor with annular tubules in a patient with Peutz-Jeghers syndrome: A case report. Mod Pathol 2000;13:466-70. |
| 5. | Connolly DC, Katabuchi H, Cliby WA, Cho KR. Somatic mutations in the STK11/LKB1 gene are uncommon in rare gynecological tumor types associated with Peutz–Jegher's syndrome. Am J Pathol 2000;156:339-45. |
[Figure 1], [Figure 2]
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