Limits...
Postauricular congenital alveolar rhabdomyosarcoma- a case report of an unusual entity.

Vankalakunti M, Das A, Rao NK - Diagn Pathol (2006)

Bottom Line: Less than 25% of patients present with evidence of cutaneous metastasis.We report a case of congenital alveolar rhabdomyosarcoma in an 18-month-old male who presented with a progressively increasing mass lesion in the left post-auricular region since birth.Radiological examination did not show any intracranial involvement of the mass lesion.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Histopathology, Postgraduate Institute of Medical Education & Research, Chandigarh, India. vkmahesh123@yahoo.co.in

ABSTRACT

Background: Congenital alveolar rhabdomyosarcoma is an extremely uncommon and invariably fatal tumor with the current therapy. Less than 25% of patients present with evidence of cutaneous metastasis.

Case presentation: We report a case of congenital alveolar rhabdomyosarcoma in an 18-month-old male who presented with a progressively increasing mass lesion in the left post-auricular region since birth. Radiological examination did not show any intracranial involvement of the mass lesion. Upon resecting the mass that was 10-cm in largest dimension, the gross, microscopic, and ultrastructural findings were consistent with congenital alveolar rhabdomyosarcoma.

Conclusion: The suspicion of alveolar subtype on histological grounds and proper evaluation of this tumor by immunostain and ultrastuctural examination is necessary. In the Medline literature search, there is no report of large congenital alveolar rhabdomyosarcoma in the post-auricle region.

No MeSH data available.


Related in: MedlinePlus

Photomicrograph showing nodules of tumor cells separated by hyalinised fibrous septae (50×, HE stain). Inset: Discohesive large tumor cells with hyperchromatic nucleus and scant cytoplasm (200×, HE stain).
© Copyright Policy - open-access
Related In: Results  -  Collection

License
getmorefigures.php?uid=PMC1621081&req=5

Figure 3: Photomicrograph showing nodules of tumor cells separated by hyalinised fibrous septae (50×, HE stain). Inset: Discohesive large tumor cells with hyperchromatic nucleus and scant cytoplasm (200×, HE stain).

Mentions: Microscopically, it revealed a varying degree of cellularity with densely packed cellular areas separated by a framework of hyalinized fibrous septa. The tumor cells were discohesive in the centre. The individual cells were large, round with darkly staining hyperchromatic nuclei, inconspicuous nucleoli, and scant indistinct cytoplasm (Figure 3). High mitotic rate was noticed. The tumor cells (≈40%) were positive for desmin and myogenin (Figure 4) immunostains. The other panels of immunostains used were CD45, CD20, MIC-2, CK, Neuron specific enolase, and myeloperoxidase, all of which were negative.


Postauricular congenital alveolar rhabdomyosarcoma- a case report of an unusual entity.

Vankalakunti M, Das A, Rao NK - Diagn Pathol (2006)

Photomicrograph showing nodules of tumor cells separated by hyalinised fibrous septae (50×, HE stain). Inset: Discohesive large tumor cells with hyperchromatic nucleus and scant cytoplasm (200×, HE stain).
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC1621081&req=5

Figure 3: Photomicrograph showing nodules of tumor cells separated by hyalinised fibrous septae (50×, HE stain). Inset: Discohesive large tumor cells with hyperchromatic nucleus and scant cytoplasm (200×, HE stain).
Mentions: Microscopically, it revealed a varying degree of cellularity with densely packed cellular areas separated by a framework of hyalinized fibrous septa. The tumor cells were discohesive in the centre. The individual cells were large, round with darkly staining hyperchromatic nuclei, inconspicuous nucleoli, and scant indistinct cytoplasm (Figure 3). High mitotic rate was noticed. The tumor cells (≈40%) were positive for desmin and myogenin (Figure 4) immunostains. The other panels of immunostains used were CD45, CD20, MIC-2, CK, Neuron specific enolase, and myeloperoxidase, all of which were negative.

Bottom Line: Less than 25% of patients present with evidence of cutaneous metastasis.We report a case of congenital alveolar rhabdomyosarcoma in an 18-month-old male who presented with a progressively increasing mass lesion in the left post-auricular region since birth.Radiological examination did not show any intracranial involvement of the mass lesion.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Histopathology, Postgraduate Institute of Medical Education & Research, Chandigarh, India. vkmahesh123@yahoo.co.in

ABSTRACT

Background: Congenital alveolar rhabdomyosarcoma is an extremely uncommon and invariably fatal tumor with the current therapy. Less than 25% of patients present with evidence of cutaneous metastasis.

Case presentation: We report a case of congenital alveolar rhabdomyosarcoma in an 18-month-old male who presented with a progressively increasing mass lesion in the left post-auricular region since birth. Radiological examination did not show any intracranial involvement of the mass lesion. Upon resecting the mass that was 10-cm in largest dimension, the gross, microscopic, and ultrastructural findings were consistent with congenital alveolar rhabdomyosarcoma.

Conclusion: The suspicion of alveolar subtype on histological grounds and proper evaluation of this tumor by immunostain and ultrastuctural examination is necessary. In the Medline literature search, there is no report of large congenital alveolar rhabdomyosarcoma in the post-auricle region.

No MeSH data available.


Related in: MedlinePlus