Sex cord tumour with annular tubules

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Sex cord tumour with annular tubules
Micrograph of a 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.

Sex cord tumour with annular tubules - intermediate magnification

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

Sex cord tumour with annular tubules - high magnification

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.

See also

• Ovarian tumor
• Sertoli cell tumor
• Granulosa cell tumor
• Peutz-Jeghers syndrome