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Unusual multifocal intraosseous papillary intralymphatic angioendothelioma (Dabska tumor) of facial bones: a case report and review of literature.

Li B, Li Y, Tian XY, Li Z - Diagn Pathol (2013)

Bottom Line: In some areas, endothelial cells formed solid-appearing aggregates with vessel lumens.A final diagnosis of intraosseous PILA was made.To the best of our knowledge, this case is the first case of primary multifocal osseous PILA.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Pathology, The First Affiliated Hospital, Sun Yat-sen university, 58, Zhongshan Road II, Guangzhou 510080, China. lizhi@mail.sysu.edu.cn.

ABSTRACT

Unlabelled: Papillary intralymphatic angioendothelioma (PILA) or Dabska tumor is extremely rare, and often affects the skin and subcutaneous tissues of children. Since its first description by Dabska, only a few intraosseous cases have been described in the literature and none of them presents with multifocal osteolytic lesion of bones. We present a case of unusual multifocal intraosseous PILA in facial bones occurring in a 1 year 3 month old male child. Computed tomography (CT) scan revealed multifocal osteolytic lesions were located at the left zygoma, left orbital bone and right maxillary. Histologically, the lesions were ill-defined and composed of multiple delicate interconnecting vascular channels with papillae formation which projected into the lumen lined by atypical plumped endothelial cells. The vascular channels were also lined by plump cuboidal endothelial cells with focal hobnailed or "match-head" appearance. In some areas, endothelial cells formed solid-appearing aggregates with vessel lumens. By immunohistochemistry, the tumor cells were positive for CD31, CD34 and D2-40 at varying intensity. A final diagnosis of intraosseous PILA was made. To the best of our knowledge, this case is the first case of primary multifocal osseous PILA.

Virtual slides: The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1919488629100787.

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Related in: MedlinePlus

Radiographic examination of the lesions. (A) Cmputed tomography (CT) scan showed osteolytic lesions of the left orbital bone (white arrow). (B) A osteolytic lesion of the left zygomatic bone (white arrow) appeared to have an indistinct border in periphery. (C) Coronal CT scan showed multiple osseous destruction of maxillary bone (black arrow) and left zygomatic bone (white arrow). (D) Postcontrast axial CT scan in soft tissue windows revealed that an irregular mass destroyed the left zygomatic bone (white arrow).
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Figure 1: Radiographic examination of the lesions. (A) Cmputed tomography (CT) scan showed osteolytic lesions of the left orbital bone (white arrow). (B) A osteolytic lesion of the left zygomatic bone (white arrow) appeared to have an indistinct border in periphery. (C) Coronal CT scan showed multiple osseous destruction of maxillary bone (black arrow) and left zygomatic bone (white arrow). (D) Postcontrast axial CT scan in soft tissue windows revealed that an irregular mass destroyed the left zygomatic bone (white arrow).

Mentions: A 1 year 3 month old Chinese male child was referred to our pediatric department for pain and swelling on his left side of face for past 2 weeks. In the past two weeks, the baby was suffering from a gradually severe soft tissues swelling on his left face. Two days before admission to our hospital, the pain and tenderness of left face became worse. As a result, the patient was referred to our hospital for examination and treatment. There was no history of any trauma to head and neck. Physical examination showed the patient had a mild soft tissues edema on his left upper face and severe pain was elicited upon pressure. There was no fever, weight loss and no palpable lymphadenopathy or organomegaly. The laboratory results, including blood count, differential, liver and renal function, were within the normal range. A computed tomography (CT) scan of the head revealed multifocal osteolytic lesions in the facial bones, including left zygomatic bone (measuring 1.5 × 1.0 × 1.0 cm in size), left orbital bone (measuring 0.5 cm in diameter) and right maxillary bone (measuring 1.0 cm in diameter). The most of left zygoma was observed to be destroyed and associated soft tissue mass was also noted (Figure 1). The lesions showed moderate enhancement after meglumine diatrizoate injection. There was no enlarged lymph node found in head and neck. A CT scan of neck and abdomen showed no pathologic findings, particularly no lymphadenopathy could be observed. A CT guided needle biopsy was performed on left zygomatic bone initially, but histopathological examination showed pieces of fibrosis with infiltration of inflammatory cells. From the clinical and radiographic evaluations, the lesion was preoperatively diagnosed as Langerhans cell histiocytosis (eosinophilic granuloma) of bone. The patient underwent curettage of the zygomatic and maxillary lesions. Because the margin of the lesions was ill-defined, the curettage was extensive. The postoperative phase was uneventful, and no additional treatments were undertaken. The pain resolved postoperatively and the patient was on regular follow-up for 24 months after discharging from hospital. A follow-up CT scan at 6 months after surgery revealed unchanged lesion of left orbital bone and there was no sign of recurrence of tumor and lymph node enlargement.


Unusual multifocal intraosseous papillary intralymphatic angioendothelioma (Dabska tumor) of facial bones: a case report and review of literature.

Li B, Li Y, Tian XY, Li Z - Diagn Pathol (2013)

Radiographic examination of the lesions. (A) Cmputed tomography (CT) scan showed osteolytic lesions of the left orbital bone (white arrow). (B) A osteolytic lesion of the left zygomatic bone (white arrow) appeared to have an indistinct border in periphery. (C) Coronal CT scan showed multiple osseous destruction of maxillary bone (black arrow) and left zygomatic bone (white arrow). (D) Postcontrast axial CT scan in soft tissue windows revealed that an irregular mass destroyed the left zygomatic bone (white arrow).
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3852300&req=5

Figure 1: Radiographic examination of the lesions. (A) Cmputed tomography (CT) scan showed osteolytic lesions of the left orbital bone (white arrow). (B) A osteolytic lesion of the left zygomatic bone (white arrow) appeared to have an indistinct border in periphery. (C) Coronal CT scan showed multiple osseous destruction of maxillary bone (black arrow) and left zygomatic bone (white arrow). (D) Postcontrast axial CT scan in soft tissue windows revealed that an irregular mass destroyed the left zygomatic bone (white arrow).
Mentions: A 1 year 3 month old Chinese male child was referred to our pediatric department for pain and swelling on his left side of face for past 2 weeks. In the past two weeks, the baby was suffering from a gradually severe soft tissues swelling on his left face. Two days before admission to our hospital, the pain and tenderness of left face became worse. As a result, the patient was referred to our hospital for examination and treatment. There was no history of any trauma to head and neck. Physical examination showed the patient had a mild soft tissues edema on his left upper face and severe pain was elicited upon pressure. There was no fever, weight loss and no palpable lymphadenopathy or organomegaly. The laboratory results, including blood count, differential, liver and renal function, were within the normal range. A computed tomography (CT) scan of the head revealed multifocal osteolytic lesions in the facial bones, including left zygomatic bone (measuring 1.5 × 1.0 × 1.0 cm in size), left orbital bone (measuring 0.5 cm in diameter) and right maxillary bone (measuring 1.0 cm in diameter). The most of left zygoma was observed to be destroyed and associated soft tissue mass was also noted (Figure 1). The lesions showed moderate enhancement after meglumine diatrizoate injection. There was no enlarged lymph node found in head and neck. A CT scan of neck and abdomen showed no pathologic findings, particularly no lymphadenopathy could be observed. A CT guided needle biopsy was performed on left zygomatic bone initially, but histopathological examination showed pieces of fibrosis with infiltration of inflammatory cells. From the clinical and radiographic evaluations, the lesion was preoperatively diagnosed as Langerhans cell histiocytosis (eosinophilic granuloma) of bone. The patient underwent curettage of the zygomatic and maxillary lesions. Because the margin of the lesions was ill-defined, the curettage was extensive. The postoperative phase was uneventful, and no additional treatments were undertaken. The pain resolved postoperatively and the patient was on regular follow-up for 24 months after discharging from hospital. A follow-up CT scan at 6 months after surgery revealed unchanged lesion of left orbital bone and there was no sign of recurrence of tumor and lymph node enlargement.

Bottom Line: In some areas, endothelial cells formed solid-appearing aggregates with vessel lumens.A final diagnosis of intraosseous PILA was made.To the best of our knowledge, this case is the first case of primary multifocal osseous PILA.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Pathology, The First Affiliated Hospital, Sun Yat-sen university, 58, Zhongshan Road II, Guangzhou 510080, China. lizhi@mail.sysu.edu.cn.

ABSTRACT

Unlabelled: Papillary intralymphatic angioendothelioma (PILA) or Dabska tumor is extremely rare, and often affects the skin and subcutaneous tissues of children. Since its first description by Dabska, only a few intraosseous cases have been described in the literature and none of them presents with multifocal osteolytic lesion of bones. We present a case of unusual multifocal intraosseous PILA in facial bones occurring in a 1 year 3 month old male child. Computed tomography (CT) scan revealed multifocal osteolytic lesions were located at the left zygoma, left orbital bone and right maxillary. Histologically, the lesions were ill-defined and composed of multiple delicate interconnecting vascular channels with papillae formation which projected into the lumen lined by atypical plumped endothelial cells. The vascular channels were also lined by plump cuboidal endothelial cells with focal hobnailed or "match-head" appearance. In some areas, endothelial cells formed solid-appearing aggregates with vessel lumens. By immunohistochemistry, the tumor cells were positive for CD31, CD34 and D2-40 at varying intensity. A final diagnosis of intraosseous PILA was made. To the best of our knowledge, this case is the first case of primary multifocal osseous PILA.

Virtual slides: The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1919488629100787.

Show MeSH
Related in: MedlinePlus