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Background <p>Primordial Odontogenic Tumor (POT) is a recently recognized entity classified under Benign Mixed Odontogenic Tumors. To date, only about 30 cases have been reported worldwide. Radiographically, it typically presents as a unilocular radiolucent lesionassociated with an unerupted molar, often resembling a dentigerous cyst. Histologically, POT comprises primitive myxoid connective tissue lined by ameloblastlike tall columnar cells, warranting a differential diagnosis that includes dentigerouscyst, unicystic ameloblastoma, ameloblastic fibroma, and odontogenic myxoma.</p> Case Report <p>We report a case of POT in a six-year-old girl presenting with swelling in the right mandibular angle and ramus region. Radiographically, the orthopantomogram revealed a well-defined, round radiolucent lesion surrounding the crown of tooth 46. On sectioning, the lesion exhibited a glistening surface. Histologically, the tumor consisted of spindle to stellate cells within a myxoid extracellular matrix resembling dental papilla. Immunohistochemically, the lesion showed positivity for CK19, CK14, p53, Vimentin, and Syndecan-1, with a low Ki-67 proliferative index. These microscopic andimmunohistochemical findings support its origin during the early stages of tooth development.</p> Outcome <p>We comprehensively analyzed the available literature on POT to better define itshistogenesis and biological behavior. The aim is to distinguish POT from otherodontogenic cysts and tumors to ensure accurate diagnosis and prevent unnecessaryaggressive treatment.</p>

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Primordial Odontogenic Tumor in Children: A Rare Entity and the Fifth Reported Case from India

  • 期刊论文

  • 发表日期：

    2026年2月19日

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  • Bhushan Sharma,
  • George Koshy,
  • Savneet Rattan,
  • Yogaen Vohra

摘要

Background

Primordial Odontogenic Tumor (POT) is a recently recognized entity classified under Benign Mixed Odontogenic Tumors. To date, only about 30 cases have been reported worldwide. Radiographically, it typically presents as a unilocular radiolucent lesionassociated with an unerupted molar, often resembling a dentigerous cyst. Histologically, POT comprises primitive myxoid connective tissue lined by ameloblastlike tall columnar cells, warranting a differential diagnosis that includes dentigerouscyst, unicystic ameloblastoma, ameloblastic fibroma, and odontogenic myxoma.

Case Report

We report a case of POT in a six-year-old girl presenting with swelling in the right mandibular angle and ramus region. Radiographically, the orthopantomogram revealed a well-defined, round radiolucent lesion surrounding the crown of tooth 46. On sectioning, the lesion exhibited a glistening surface. Histologically, the tumor consisted of spindle to stellate cells within a myxoid extracellular matrix resembling dental papilla. Immunohistochemically, the lesion showed positivity for CK19, CK14, p53, Vimentin, and Syndecan-1, with a low Ki-67 proliferative index. These microscopic andimmunohistochemical findings support its origin during the early stages of tooth development.

Outcome

We comprehensively analyzed the available literature on POT to better define itshistogenesis and biological behavior. The aim is to distinguish POT from otherodontogenic cysts and tumors to ensure accurate diagnosis and prevent unnecessaryaggressive treatment.