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Table 1 Diagnostic criteria for Peutz Jeghers Syndrome (PJS)

From: High-grade endometrial stromal sarcoma as the initial presentation of an adult patient with Peutz-Jeghers Syndrome: a case report

A. In patients without a family history of PJS, one of the following must be present: • Characteristic melanotic macules and one or more intestinal polyps with PJS-type histology*, or
• Two intestinal polyps with PJS-type histology*
B. In patients with a family history of PJS in a first degree relative, one of the following must be present: • Characteristic melanotic macules, or
• One intestinal polyp with PJS-type histology*, or
STK11 mutation