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Pediatric Metastatic Odontogenic Ghost Cell Carcinoma: A Multimodal Treatment Approach.

Ahmed SK, Watanabe M, deMello DE, Daniels TB - Rare Tumors (2015)

Bottom Line: Odontogenic ghost cell carcinoma (OGCC) is a rare and aggressive tumor wherein optimal treatment remains uncertain.Adjuvant therapy was utilized due to locally advanced disease with pathologic features indicative of high recurrence risk.Our patient is alive and disease free at 14 months indicating a potentially positive role for multimodal therapy in the management of OGCC.

View Article: PubMed Central - PubMed

Affiliation: Department of Radiation Oncology, Mayo Clinic , Rochester, MN.

ABSTRACT
Odontogenic ghost cell carcinoma (OGCC) is a rare and aggressive tumor wherein optimal treatment remains uncertain. We report the first pediatric metastatic OGCC case treated with multimodal therapy: surgery, adjuvant chemoradiation, and adjuvant immunotherapy. Adjuvant therapy was utilized due to locally advanced disease with pathologic features indicative of high recurrence risk. This multimodal approach was modeled after management of primary head and neck cancer, where adjuvant chemoradiation and immunotherapy are associated with improved outcomes. Our patient is alive and disease free at 14 months indicating a potentially positive role for multimodal therapy in the management of OGCC.

No MeSH data available.


Related in: MedlinePlus

Islands of squamous epithelial cells with foci of ghost cells and calcification (Hematoxylin & Eosin, 400×).
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fig001: Islands of squamous epithelial cells with foci of ghost cells and calcification (Hematoxylin & Eosin, 400×).

Mentions: A 10 year-old Hispanic male presented with a fluctuant mass in the right maxilla. Computed tomography (CT) scan revealed a 3.3 cm soft tissue lesion with destruction of the adjacent maxillary bone and sinus, and displacement of several teeth. Biopsy demonstrated OGCC. Preoperative staging five weeks later revealed size progression and metastatic disease. The mass now measured 5.3 cm. A right submandibular node and several enlarged right cervical lymph nodes were noted corresponding to FDG avidity on PET. Right-sided modified radical maxillectomy and palatectomy, and right-sided modified radical neck dissection was performed one week later. Significant involvement of the buccal mucosa and submandibular gland were noted. After multiple attempts, final surgical margins were negative. Pathology confirmed OGCC (Figure 1). Seven of thirty-eight Level I and II lymph nodes were positive for metastases. The tumor was positive for EGF receptor indicating cellular expression of EGFR protein. One week after surgery, maxillary packing was removed in the operating room. No tumor recurrence was appreciated. Adjuvant therapy was recommended given the rapid pre-surgery growth, positive lymph nodes, and concern for microscopic residual disease. A lesion in the right buccal space was noted at the time of radiation simulation (19 days after surgery). Given these findings and prior history of rapid growth, this was presumed recurrent tumor. An intensity modulated radiation therapy (IMRT) plan was designed for the entire rightsided postoperative bed and bilateral neck. The patient received 44 Gray in 22 fractions (6 mega-Volt photons) prior to receiving a break for treatment-related toxicities. Imaging obtained then showed a 4.9 cm soft tissue mass in the right cheek and a 1.7 cm left gingivolabial soft tissue mass (Figure 2). Biopsy confirmed OGCC. Radiation volumes were expanded to include the new disease.


Pediatric Metastatic Odontogenic Ghost Cell Carcinoma: A Multimodal Treatment Approach.

Ahmed SK, Watanabe M, deMello DE, Daniels TB - Rare Tumors (2015)

Islands of squamous epithelial cells with foci of ghost cells and calcification (Hematoxylin & Eosin, 400×).
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4508645&req=5

fig001: Islands of squamous epithelial cells with foci of ghost cells and calcification (Hematoxylin & Eosin, 400×).
Mentions: A 10 year-old Hispanic male presented with a fluctuant mass in the right maxilla. Computed tomography (CT) scan revealed a 3.3 cm soft tissue lesion with destruction of the adjacent maxillary bone and sinus, and displacement of several teeth. Biopsy demonstrated OGCC. Preoperative staging five weeks later revealed size progression and metastatic disease. The mass now measured 5.3 cm. A right submandibular node and several enlarged right cervical lymph nodes were noted corresponding to FDG avidity on PET. Right-sided modified radical maxillectomy and palatectomy, and right-sided modified radical neck dissection was performed one week later. Significant involvement of the buccal mucosa and submandibular gland were noted. After multiple attempts, final surgical margins were negative. Pathology confirmed OGCC (Figure 1). Seven of thirty-eight Level I and II lymph nodes were positive for metastases. The tumor was positive for EGF receptor indicating cellular expression of EGFR protein. One week after surgery, maxillary packing was removed in the operating room. No tumor recurrence was appreciated. Adjuvant therapy was recommended given the rapid pre-surgery growth, positive lymph nodes, and concern for microscopic residual disease. A lesion in the right buccal space was noted at the time of radiation simulation (19 days after surgery). Given these findings and prior history of rapid growth, this was presumed recurrent tumor. An intensity modulated radiation therapy (IMRT) plan was designed for the entire rightsided postoperative bed and bilateral neck. The patient received 44 Gray in 22 fractions (6 mega-Volt photons) prior to receiving a break for treatment-related toxicities. Imaging obtained then showed a 4.9 cm soft tissue mass in the right cheek and a 1.7 cm left gingivolabial soft tissue mass (Figure 2). Biopsy confirmed OGCC. Radiation volumes were expanded to include the new disease.

Bottom Line: Odontogenic ghost cell carcinoma (OGCC) is a rare and aggressive tumor wherein optimal treatment remains uncertain.Adjuvant therapy was utilized due to locally advanced disease with pathologic features indicative of high recurrence risk.Our patient is alive and disease free at 14 months indicating a potentially positive role for multimodal therapy in the management of OGCC.

View Article: PubMed Central - PubMed

Affiliation: Department of Radiation Oncology, Mayo Clinic , Rochester, MN.

ABSTRACT
Odontogenic ghost cell carcinoma (OGCC) is a rare and aggressive tumor wherein optimal treatment remains uncertain. We report the first pediatric metastatic OGCC case treated with multimodal therapy: surgery, adjuvant chemoradiation, and adjuvant immunotherapy. Adjuvant therapy was utilized due to locally advanced disease with pathologic features indicative of high recurrence risk. This multimodal approach was modeled after management of primary head and neck cancer, where adjuvant chemoradiation and immunotherapy are associated with improved outcomes. Our patient is alive and disease free at 14 months indicating a potentially positive role for multimodal therapy in the management of OGCC.

No MeSH data available.


Related in: MedlinePlus