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Primary peripheral primitive neuroectodermal tumor of the mandible.

Krishnamurthy A, Ramshankar V, Majhi U, George R - Ann Maxillofac Surg (2013)

Bottom Line: A review of the scientific literature describes only seven reports of pPNET to be arising from the mandible.There is however no consensus about the best therapeutic strategy.We recently got to treat a young patient with mandibular PNET; the clinical course as well as the histopathology with immunohistochemistry correlation of this rare entity is discussed.

View Article: PubMed Central - PubMed

Affiliation: Department of Surgical Oncology, Cancer Institute (Women's India Association), Adyar, Chennai, Tamil Nadu, India.

ABSTRACT
Primitive neuroectodermal tumor (PNET) is a broad term that includes a wide array of lesions with varying differentiating potential affecting both the central and peripheral nervous system. Peripheral PNET (pPNET), a variant of PNET, is known to arise in many places throughout the body; involvement of the mandible is however rare. A review of the scientific literature describes only seven reports of pPNET to be arising from the mandible. Given their insidious clinical symptoms, variable locations and rarity, the accurate diagnosis of peripheral PNETs poses a challenge for clinicians. PNETs exhibits characteristic immunophenotypical and genetic features that distinguish them from other small round cell tumors. A multidisciplinary approach is necessary to manage patients affected by PNET. There is however no consensus about the best therapeutic strategy. We recently got to treat a young patient with mandibular PNET; the clinical course as well as the histopathology with immunohistochemistry correlation of this rare entity is discussed.

No MeSH data available.


Related in: MedlinePlus

CT scan: (a) Axial section, (b) Coronal section, and (c) Reconstructed image showing a heterodense mass lesion measuring 8 × 7 cm at its greatest dimension, causing bony destruction of the ascending mandibular ramus, with extension into the skull base. The tumor caused effacement of the fat and muscle planes of the infratemporal fossa and the masticator space, (d) A bone scintigram showed an increased abnormal activity in the left side of the mandible corresponding to the tumor
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Figure 2: CT scan: (a) Axial section, (b) Coronal section, and (c) Reconstructed image showing a heterodense mass lesion measuring 8 × 7 cm at its greatest dimension, causing bony destruction of the ascending mandibular ramus, with extension into the skull base. The tumor caused effacement of the fat and muscle planes of the infratemporal fossa and the masticator space, (d) A bone scintigram showed an increased abnormal activity in the left side of the mandible corresponding to the tumor

Mentions: Computed tomography (CT) revealed a heterodense mass lesion measuring 8 × 7 cm at its greatest dimension causing bony destruction of the ascending mandibular ramus and with extension into the skull base. The tumor caused effacement of the fat and muscle planes of the infratemporal fossa and the masticator spaces. It was seen pushing the parotid space laterally and the parapharyngeal space medially. The left temporomandibular joint was obliterated by the tumor [Figures 2a–c]. A bone scintigram showed an increased abnormal activity in the left side of mandible corresponding to the tumor [Figure 2d]. A bone marrow aspiration and a bone marrow biopsy were within normal limits. An extraoral trucut biopsy from the tumor on histopathologic examination revealed a diffused collection of small malignant round to oval cells with hyperchromatic nuclei, inconspicuous nucleoli, with vacuolated to pale cytoplasm and high mitotic activity, and scattered blood vessels with peritheliomatous arrangements of the tumor cells. The tumor cells on immunohistochemistry were positive for vimentin, CD99, Bcl2, chromogranin, and S-100. It was negative for keratin, LCA, CD3, CD 20, CD56, and Alk 1. Fifty percentage of the tumor cells showed strong nuclear positivity to Ki-67. The histopathology along with immunohistochemistry correlation favored a diagnosis of PNET of the mandible [Figures 3a–d].


Primary peripheral primitive neuroectodermal tumor of the mandible.

Krishnamurthy A, Ramshankar V, Majhi U, George R - Ann Maxillofac Surg (2013)

CT scan: (a) Axial section, (b) Coronal section, and (c) Reconstructed image showing a heterodense mass lesion measuring 8 × 7 cm at its greatest dimension, causing bony destruction of the ascending mandibular ramus, with extension into the skull base. The tumor caused effacement of the fat and muscle planes of the infratemporal fossa and the masticator space, (d) A bone scintigram showed an increased abnormal activity in the left side of the mandible corresponding to the tumor
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3814671&req=5

Figure 2: CT scan: (a) Axial section, (b) Coronal section, and (c) Reconstructed image showing a heterodense mass lesion measuring 8 × 7 cm at its greatest dimension, causing bony destruction of the ascending mandibular ramus, with extension into the skull base. The tumor caused effacement of the fat and muscle planes of the infratemporal fossa and the masticator space, (d) A bone scintigram showed an increased abnormal activity in the left side of the mandible corresponding to the tumor
Mentions: Computed tomography (CT) revealed a heterodense mass lesion measuring 8 × 7 cm at its greatest dimension causing bony destruction of the ascending mandibular ramus and with extension into the skull base. The tumor caused effacement of the fat and muscle planes of the infratemporal fossa and the masticator spaces. It was seen pushing the parotid space laterally and the parapharyngeal space medially. The left temporomandibular joint was obliterated by the tumor [Figures 2a–c]. A bone scintigram showed an increased abnormal activity in the left side of mandible corresponding to the tumor [Figure 2d]. A bone marrow aspiration and a bone marrow biopsy were within normal limits. An extraoral trucut biopsy from the tumor on histopathologic examination revealed a diffused collection of small malignant round to oval cells with hyperchromatic nuclei, inconspicuous nucleoli, with vacuolated to pale cytoplasm and high mitotic activity, and scattered blood vessels with peritheliomatous arrangements of the tumor cells. The tumor cells on immunohistochemistry were positive for vimentin, CD99, Bcl2, chromogranin, and S-100. It was negative for keratin, LCA, CD3, CD 20, CD56, and Alk 1. Fifty percentage of the tumor cells showed strong nuclear positivity to Ki-67. The histopathology along with immunohistochemistry correlation favored a diagnosis of PNET of the mandible [Figures 3a–d].

Bottom Line: A review of the scientific literature describes only seven reports of pPNET to be arising from the mandible.There is however no consensus about the best therapeutic strategy.We recently got to treat a young patient with mandibular PNET; the clinical course as well as the histopathology with immunohistochemistry correlation of this rare entity is discussed.

View Article: PubMed Central - PubMed

Affiliation: Department of Surgical Oncology, Cancer Institute (Women's India Association), Adyar, Chennai, Tamil Nadu, India.

ABSTRACT
Primitive neuroectodermal tumor (PNET) is a broad term that includes a wide array of lesions with varying differentiating potential affecting both the central and peripheral nervous system. Peripheral PNET (pPNET), a variant of PNET, is known to arise in many places throughout the body; involvement of the mandible is however rare. A review of the scientific literature describes only seven reports of pPNET to be arising from the mandible. Given their insidious clinical symptoms, variable locations and rarity, the accurate diagnosis of peripheral PNETs poses a challenge for clinicians. PNETs exhibits characteristic immunophenotypical and genetic features that distinguish them from other small round cell tumors. A multidisciplinary approach is necessary to manage patients affected by PNET. There is however no consensus about the best therapeutic strategy. We recently got to treat a young patient with mandibular PNET; the clinical course as well as the histopathology with immunohistochemistry correlation of this rare entity is discussed.

No MeSH data available.


Related in: MedlinePlus