Peutz-Jeghers syndrome with feminizing sertoli cell tumor

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Recurrent ovarian Sertoli–Leydig cell tumor in a child with Peutz–Jeghers syndrome

We present a female child with Peutz-Jeghers syndrome (PJS) with a recurrent ovarian Sertoli-Leydig cell tumor (SLCT). SLCTs are relatively rare sex cord neoplasms that can occur in PJS. The patient was an African-American female who first presented at the age of 3 years with precocious puberty, and then at the age of 17 years with abdominal pain and irregular menses. In each case, she had rese...

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Malignant Large Cell Calcifying Sertoli Cell Tumor of Testis with Skip Metastasis to Lung Presented With Peutz-Jeghers Syndrome

Large cell calcifying Sertoli cell tumor of the testis (LCCSCT) is a rare tumor that is usually benign and multifocal. It may be associated with hereditary endocrine anomalies such as Carney's and Peutz-Jeghers syndromes. It is a rare histological variant of sex cord stromal tumors. It is exceptional in elderly men and the outcome is rarely fatal. We report a case of LCCSCT in a 44 year-old man...

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Response to Anastrozole Treatment in a Case with Peutz-Jeghers Syndrome and a Large Cell Calcifying Sertoli Cell Tumor

Peutz-Jeghers syndrome (PJS) is inherited as an autosomal dominant trait characterized by multiple gastrointestinal hamartomatous polyps, mucocutaneous pigmentation, and an increased risk of neoplasm. Large-cell calcifying Sertoli cell tumor (LCCSCT) is a kind of sex cord-stromal tumor which may co-exist with PJS and which is characterized radiologically by calcification foci within the testes....

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Peutz-Jeghers syndrome

Introduction Peutz-Jeghers syndrome (PJS) is characterized by: (i) autosomal dominant inheritance; (ii) cutaneous pigmentation; (iii) gastro-intestinal polyposis. In all, more than 300 cases have been described with a world-wide distribution and no racial predilection. In 1921 Peutz described 7 cases of multiple intestinal polyps associated with melanin spots on the lips, buccal mucosa and digi...

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[Peutz-Jeghers syndrome].

Peutz-Jeghers syndrome is an inherited disorder which usually debuts during childhood. It is characterized by mucocutaneous pigmentation and hamartomatous polyps in the gastrointestinal tract. Numerous reports indicate a high incidence of gastrointestinal and extraintestinal cancer in these patients, their appearance at a young age, as well as its association with ovarian and testicular tumors....

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ژورنال

عنوان ژورنال: Cancer

سال: 1980

ISSN: 0008-543X,1097-0142

DOI: 10.1002/1097-0142(19800701)46:1<223::aid-cncr2820460137>3.0.co;2-8