Sex cord tumour with annular tubules
Editor-In-Chief: Prab R Tumpati, MD
Obesity, Sleep & Internal medicine
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| Sex cord tumour with annular tubules | |
|---|---|
| Synonyms | SCAT |
| Pronounce | N/A |
| Specialty | N/A |
| Symptoms | Often asymptomatic, may present with abdominal pain or pelvic mass |
| Complications | Infertility, endometrial hyperplasia |
| Onset | Typically in adulthood |
| Duration | Variable |
| Types | Benign, malignant |
| Causes | Unknown |
| Risks | Peutz-Jeghers syndrome |
| Diagnosis | Histopathology |
| Differential diagnosis | Granulosa cell tumour, Sertoli cell tumour |
| Prevention | N/A |
| Treatment | Surgical resection |
| Medication | None specific |
| Prognosis | Generally good for benign cases |
| Frequency | Rare |
| Deaths | N/A |
A sex cord tumour with annular tubules (SCTAT) is a rare type of ovarian tumor that exhibits features of both Sertoli cell and granulosa cell tumors. It is characterized by distinctive histological patterns, including annular tubules, which are ring-like structures formed by the tumor cells.
Histopathology
The hallmark of SCTAT is the presence of annular tubules. These structures are composed of columnar or cuboidal cells arranged in a circular pattern around a central hyaline core. The tubules can vary in size and may be solid or have a lumen. The cells typically have moderate amounts of cytoplasm and round to oval nuclei.
SCTATs are often associated with Peutz-Jeghers syndrome, a genetic condition characterized by gastrointestinal polyps and mucocutaneous pigmentation. In patients with this syndrome, SCTATs are usually bilateral and multifocal.
Clinical Presentation
Patients with SCTAT may present with abdominal pain or a palpable mass. In some cases, hormonal activity of the tumor can lead to symptoms such as precocious puberty in children or menstrual irregularities in adults. The association with Peutz-Jeghers syndrome may also lead to the identification of SCTAT during the evaluation of other symptoms related to the syndrome.
Diagnosis
Diagnosis of SCTAT is primarily based on histological examination of the tumor tissue. Imaging studies such as ultrasound or MRI may be used to evaluate the extent of the tumor and its characteristics.
Treatment
The primary treatment for SCTAT is surgical removal of the tumor. The extent of surgery depends on the size and spread of the tumor, as well as the patient's age and desire for fertility preservation. In cases associated with Peutz-Jeghers syndrome, careful monitoring and management of other syndrome-related complications are also necessary.
Prognosis
The prognosis for patients with SCTAT is generally favorable, especially when the tumor is confined to the ovary and completely resected. However, the presence of Peutz-Jeghers syndrome may complicate the overall clinical picture due to the risk of other malignancies associated with the syndrome.
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Contributors: Prab R. Tumpati, MD