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Year : 2023  |  Volume : 66  |  Issue : 1  |  Page : 202-204
Pediatric intranasal lobular capillary hemangioma


1 Department of ENT, Christian Medical College, Vellore, Tamil Nadu, India
2 Department of Pathology, Christian Medical College, Vellore, Tamil Nadu, India

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Date of Submission12-Jun-2021
Date of Decision05-Jul-2021
Date of Acceptance06-Jul-2021
Date of Web Publication18-Jan-2023
 

How to cite this article:
Naina P, Nithya R, Eapen P. Pediatric intranasal lobular capillary hemangioma. Indian J Pathol Microbiol 2023;66:202-4

How to cite this URL:
Naina P, Nithya R, Eapen P. Pediatric intranasal lobular capillary hemangioma. Indian J Pathol Microbiol [serial online] 2023 [cited 2023 Feb 7];66:202-4. Available from: https://www.ijpmonline.org/text.asp?2023/66/1/202/367965





   Clinical History Top


A 5-year-old girl was referred to the pediatric otolaryngology clinic with a 1 month history of left-sided significant unprovoked epistaxis, bilateral nasal block, and left facial pain. Nasal endoscopy showed a reddish fleshy mass filling the left nasal cavity pushing the septum to right [Figure 1]a. Contrast-enhanced computed tomography of the paranasal sinus revealed a large fairly well-defined heterogeneous enhancing lesion in left nasal cavity with areas of necrosis and calcification. The lesion was extending laterally causing erosion of medial wall of maxillary sinus and ethmoid bone [Figure 1]b. Biopsy was subjected to histopathological and immunohistochemistry examination [Figure 2], [Figure 3], [Figure 4].
Figure 1: (a) Endoscopic image showing a large fleshy mass up to the level of the inferior turbinate. (b) (inset) Coronal image of contrast-enhanced computed tomography showing a large heterogeneous enhancing mass with areas of calcification. Erosion of medial wall of left maxillary sinus with retained secretions in all sinuses can be noted

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Figure 2: Multiple proliferated vascular channels with adjacent spindle cells with mildly pleomorphic nucleus, dispersed chromatin, and inconspicuous nucleoli (H and E, ×400)

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Figure 3: Immunohistochemistry showing spindle cells positive for SMA. (×400)

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Figure 4: Immunohistochemistry showing blood vessels are positive for CD34 highlighting the vascular channels (×400)

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   Microscopic Findings Top


Histopathological examination of the lesion showed fibrocollageneous tissue with multiple proliferated and dilated blood vessels along with spindle cells [Figure 2]. The adjacent stroma showed collagenization, hyalinization, extravasated Red blood cells (RBC) and mild infiltrates of lymphocytes and histiocytes. The mitotic activity was low (1-2/10 High power fields (HPF)) and there were no areas of necrosis [Figure 2]. On immunohistochemistry, the spindle cells are positive for smooth muscle actin (SMA). CD 34 highlights the benign endothelial cells of vessels. The spindle cells are negative for beta-catenin, myogenin, androgen receptor (AR), ALK, and STAT6 [Figure 3] and [Figure 4].

  1. What is your diagnosis?
  2. What are your differentials?


Answer

  1. Intranasal lobular capillary haemangioma
  2. Juvenile nasopharyngeal angiofibroma - Unlikely in females and AR negative


b. Angiosarcoma- Not likely as benign histopathology, low mitotic figure, mildly pleomorphic. CD34 was negative in the spindle cells.

d. Rhabdomyosarcoma- Unlikely due to lack of rhabdoid like morphology. Desmin was negative in the spindle cells.

e. Inflammatory myofibroblastic tumor- Lack of much inflammation. ALK1 was negative in the spindle cells.


   Discussion Top


Intranasal lobular capillary hemangiomas are unusual vascular tumors in the pediatric age group with less than 20 case reports in literature in children.[1],[2],[3] They are more common in the third and fifth decade of life where larger series have been reported.[4],[5],[6] The exact etiopathogenesis remains unknown though local trauma and hormonal influences are considered to play a major role. The contributory role of viral oncogenes and angiogenic growth factors has also been proposed.[1],[2],[4],[5],[6] Various attempts to explain the pathogenesis has led to various synonyms for these tumors, such as botryomycose humane, granuloma pyogenicum, pregnancy tumor, granulosa gravidarum, and telengieactic polyp. This nomenclature is proposed based on the close hormonal association between pregnancy and LCH in adults.[4],[5],[6] The term LCH was coined in 1980 due to it typical histological features of extensive endothelial proliferation with prominent vascular spaces, lobular arrangement of capillaries, and a fibrovascular core. The common clinical presentation in children is epistaxis, nose block, and mouth breathing. The differential diagnosis for a vascular nasal mass in children include angiofibroma, angiomatous polyps, gliomas, histiocytoma, or malignant tumors, such as aesthesioneuroblastoma, angiosarcoma, rhabdomyosarcoma, Kaposi sarcoma, lymphoma, or adenocarcinoma.[1],[2],[4],[5] Contrast-enhanced computed tomography is the investigation of choice showing an expansile well-enhancing heterogeneous mass with bony remodeling and internal calcification.[7] The characteristic finding of hemangioma is seen on T2-weighted Magnetic resonance imaging (MRI) images as a vascular mass with multiple flow voids surrounding an inner matrix of hyper intense tissue.[1],[2],[7] Hemangiomas generally maintain bone architecture but in case of a large tumor in a small child there can be bone erosion mimicking malignancy.[4] Complete surgical excision is the treatment of choice and the most preferred approach is the endoscopic approach due to better visualization, less morbidity, and good cosmetic outcome.[1],[2],[4],[5],[6] In certain situation, such as a small child, large deep seated tumors, and pregnancy, preoperative embolization can help reduce blood loss.[6]

This case is worth highlighting as typical clinical and radiological features of malignancy can present in large intranasal LCH but histopathology and immunohistochemistry can confirm diagnosis.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Mariño-Sánchez F, Lopez-Chacon M, Jou C, Haag O. Pediatric intranasal lobular capillary hemangioma: Report of two new cases and review of the literature. Respir Med Case Rep 2016;18:31-4.  Back to cited text no. 1
    
2.
Virbalas JM, Bent JP, Parikh SR. Pediatric nasal lobular capillary hemangioma. Case Rep Med 2012;2012:769630.  Back to cited text no. 2
    
3.
Yıldırım U, Karlı R, Gün S. Pediatric intranasal lobular capillary hemangioma: A rare clinical entity. Balkan Med J 2017;34:586-7.  Back to cited text no. 3
    
4.
Alghamdi B, Al-Kadi M, Alkhayal N, Alhedaithy R, Al Mahdi MJ. Intranasal lobular capillary hemangioma: A series of five cases. Respir Med Case Rep 2020;30:101073.  Back to cited text no. 4
    
5.
Puxeddu R, Berlucchi M, Ledda GP, Parodo G, Farina D, Nicolai P. Lobular capillary hemangioma of the nasal cavity: A retrospective study on 40 patients. Am J Rhinol 2006;20:480-4.  Back to cited text no. 5
    
6.
Takaishi S, Asaka D, Nakayama T, Iimura J, Matsuwaki Y, Hirooka S, et al. Features of sinonasalhemangioma: A retrospective study of 31 cases. Auris Nasus Larynx 2017;44:719-23.  Back to cited text no. 6
    
7.
Yang BT, Li SP, Wang YZ, Dong JY, Wang ZC. Routine and dynamic MR imaging study of lobular capillary hemangioma of the nasal cavity with comparison to inverting papilloma. AJNR Am J Neuroradiol 2013;34:2202-7.  Back to cited text no. 7
    

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Correspondence Address:
P Naina
Department of ENT, Christian Medical College, Vellore - 632 004, Tamil Nadu
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijpm.ijpm_592_21

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    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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