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Year : 2019  |  Volume : 15  |  Issue : 3  |  Page : 729-732

Hemangiopericytoma of supraglottis: A rare case report and review of literature

1 Department of Medical and Paediatric Oncology, Gujarat and Cancer Research Institute, Ahmedabad, Gujarat, India
2 Department of Radiology, Gujarat and Cancer Research Institute, Ahmedabad, Gujarat, India
3 Department of Pathology, Gujarat and Cancer Research Institute, Ahmedabad, Gujarat, India

Date of Web Publication29-May-2019

Correspondence Address:
Dr. Apurva A Patel
Department of Medical Oncology, Gujarat and Cancer Research Institute, Ahmedabad - 380 016, Gujarat
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jcrt.JCRT_939_16

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 > Abstract 

In 1942, Stout and Murray first used the term hemangiopericytoma to describe a tumor which is distinguished histologically from other types of vascular neoplasm characterized by proliferation of pericytes. It is a rare neoplasm that was originally described as a vascular tumor derived from the pericytes. They account for 2%–3% of all soft tissue sarcomas in humans and they occur mainly in the musculoskeletal system. About 15%-30% of all hemangiopericytomas occur in the head and neck region. Hemangiopericytoma of supraglottis is very rare neoplasm with only nine cases reported in literature and ours is the tenth case overall and first case in pediatric age group. Herein, we are presenting an extremely rare case report of hemangiopericytoma of supraglottis in a 6-year-old male child who presented with stridor followed by which tracheostomy was done. Later, the patient was treated initially with radiotherapy followed by surgery, i.e., laryngectomy in view of residual disease postcurative radiotherapy. Hence, hemangiopericytoma is a very rare tumor overall and can present in pediatric age group and can be one most important differential diagnosis because many patients in this age group, stridor most commonly occurs due to the infectious causes such as influenza virus and diphtheria-induced croup, i.e., laryngotracheobronchitis.

Keywords: Hemangiopericytoma, radiotherapy, supraglottis

How to cite this article:
Kendre P, Kataria P, Patel AA, Gaurav L, Dalsaniya S. Hemangiopericytoma of supraglottis: A rare case report and review of literature. J Can Res Ther 2019;15:729-32

How to cite this URL:
Kendre P, Kataria P, Patel AA, Gaurav L, Dalsaniya S. Hemangiopericytoma of supraglottis: A rare case report and review of literature. J Can Res Ther [serial online] 2019 [cited 2021 Nov 27];15:729-32. Available from: https://www.cancerjournal.net/text.asp?2019/15/3/729/209963

 > Introduction Top

Hemangiopericytoma is rare perivascular tumor developing as a result of the uncontrolled proliferation of capillary pericytes. Pericytes are rudimentary cells that have contractile proteins that regulate blood flow through capillaries. Hemangiopericytoma develops in deep soft tissues, especially those of the extremities or retroperitoneum, and commonly affects middle-aged patients.[1] However, the occurrence of hemangiopericytoma of head and neck and that to supraglottis is rare. Due to the rarity of this tumor, the clinical course, treatment outcomes, prognostic factors, and need for adjunctive treatment have not been well delineated. Hence, were hereby report a rare occurrence of hemangiopericytoma of supraglottis in a 6-year-old child who was treated with radiotherapy followed by surgery.

 > Case Report Top

A 6-year-old child presented to us one evening to the emergency department with sudden onset stridor. The patient was admitted and was given oxygen support with intravenous normal saline for hydration meanwhile patient was being prepared for tracheostomy. The patient had undergone the routine investigation like complete blood count and the serum renal and liver chemistry at a local hospital 2 days back. On reviewing the reports and with high-risk consent, the patient underwent tracheostomy uneventfully and patient was kept in intensive care unit with antibiotic and oxygen support. Later on, patients' relatives gave us a history of hoarseness of voice for 1 month and dyspnea for 15 days before presentation. After initial support, the patient underwent computed tomography (CT) of the paranasal sinus and neck which showed a heterogeneously enhancing soft tissue density lesion involving the posterior hypopharyngeal wall, superiorly extending into the posterior oropharyngeal wall with significant luminal narrowing was evident. Inferiorly lesion involved bilateral aryepiglottic (AE) folds and both pyriform fossa [Figure 1]. Direct laryngoscopy under anesthesia was performed to look for the findings of the CT and to get the biopsy as well to delineate the lesion. On laryngoscopy under anesthesia, soft tissue density lesion was seen involving bilateral AE folds, both pyriform fossa. Biopsy was taken during the procedure. Biopsy report was suggestive of high-grade undifferentiated tumor showing large round cells [Figure 2] immunohistochemistry (IHC) was done on tissue biopsy which was consistent with hemangiopericytoma (Vimentin+, CD34+, CD99). Chest X-ray was normal, and other biochemical investigation were within normal limits. As we were dealing with locally advanced disease with tumor, node, metastasis stage, i.e., stage IVA, the patient was considered for curative radiotherapy, i.e., 30 Gy/15 fractions over 3–4 weeks. The patient tolerated the radiotherapy well, and there was good subjective improvement. After 4 weeks of chemotherapy, the patient underwent CT of the paranasal sinus and neck which showed residual lesion involving postcricoid region, bilateral AE fold pyriform fossa, lateral and posterior hypopharyngeal wall [Figure 3]. Biopsy was taken from the site of residual disease which was suggestive of neoplasm consistent with hemangiopericytoma. Later, the patient was planned for total laryngectomy and patient underwent the procedure without any sequelae. Since 9 months, the patient is under regular follow-up, and we had done the interval scan which was within normal limit [Figure 4].
Figure 1: Heterogeneously enhancing soft tissue density lesion involving the posterior hypopharyngeal wall, superiorly extending into the posterior oropharyngeal wall

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Figure 2: HPE (×40) of supraglottic mass showing high-grade undifferentiated large round cells

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Figure 3: Postradiotherapy computed tomography (paranasal sinuses + neck) axial view showing residual disease at postcricoid region, b/l aryepiglottic fold and pyriform fossa, lateral and posterior hypopharyngeal wall

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Figure 4: Computed tomography (paranasal sinuses + neck) axial view few months after laryngectomy with no evidence of any abnormally enhancing lesion at operated site

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 > Discussuion Top

Stout and Murray first described hemangiopericytoma in 1942.[2] Hemangiopericytoma is an uncommon tumor that constitute <1% of all neoplasm which has been thought to originate from the pericytes of Zimmermann that surround the basement membrane of capillaries.[3],[4] Most commonly it occurs in lower extremities and retroperitoneum. Other less common site includes head and neck involving soft tissues of neck, mouth, and sinonasal tract. Forty percent of hemangiopericytomas occur in the fifth and sixth decades of life, however, it can occur in all age groups. Hemangiopericytoma of supraglottis is very rare neoplasm with only nine cases reported in literature.[5]

Hemangiopericytomas can be classified as benign or malignant based on histology. Other classification is based on the age of occurrence, i.e., infantile type that occurs in the 1st year of life and the adult type which occur in adults and children older than 1 year. Infantile type is mainly congenital and located mainly in subcutis and oral cavity. They can be multifocal and may exist as metastatic disease. Response to chemotherapy is good and have favorable prognosis as compared to adult forms which are associated with poor prognosis.

Local recurrences are known to occur in malignant hemangiopericytoma with the incidence of 40%. They are also prone for metastasis and incidence has been reported to about 30%–33% in various head neck series [3],[6] with the most common sites of metastases being lungs and bone. Distant metastases have been reported between 18% and 57% in the literature and can occur uptill 11 years after initial diagnosis and treatment.[6]

Clinical symptoms are vague and nonspecific, that depends on the tumor location and size. Radiologically, they may present as either lytic or focal sclerosis, or they may also show honeycomb or reticular pattern. At times, cortical erosion on radiological imaging can alarm about the presence of malignancy. Radiological investigations such as CT and magnetic resonance images (MRIs) are nondiagnostic. Their role is to differentiate benign tumors from those which are malignant, and they point toward the extent of the tumor. For example, MRI neck plays an important role for delineating disease extension and preoperative planning. Fluorodeoxyglucose positron emission tomography may be helpful in revealing multiple distant metastases.

Other than radiological imaging which helps to gain insight about above-said features, accurate diagnosis can be made only through histopathology examination of specimen and IHC to confirm the diagnosis.

Hemangiopericytomas are highly cellular and vascularized tumors, consisting of tightly packed, round to fusiform cells, around a well-developed, elaborate branching “staghorn” vasculature.[7],[8] Staghorn appearance on histology is due to irregular vascular channels showing branches of varying sizes that give the characteristic appearance. Nucleoli are usually single and show finely dispersed chromatin. On histopathological examination, different patterns that need to be recognized from other common patterns include large mucoid interstitial appearance which can resemble mucoid type of lipoma or that of liposarcoma of myxoid type. Focal areas of cartilage production can be observed which can be confused with mesenchymal chondrosarcomas.

Biologic behavior exhibited by the hemangiopericytomas can span from being a slow growing mass to an aggressive growth pattern having characteristics of malignancy; also borderline or intermediate type of behavior can be seen with characteristic between the above two features. According to the World Health Organization classification, hepatic progenitor cells (HPCs) are graded as being differentiated (Grade II) and anaplastic (Grade III).[9] Signs of anaplasia include high mitotic rate (>5 mitoses per high-power field) and/or necrosis, plus at least two of the following features: hemorrhage, moderate to high nuclear atypia, and cellularity.[8] The other soft tissue tumors are ruled out on the basis of IHC. Immunohistochemically, tumor cells usually express CD34 and vimentin but not epithelial membrane antigen and S100.[7],[9] Immunoreactivity patterns generally vary among HPCs, and no antibody is 100% sensitive or specific.[9] The differential diagnosis includes both benign (fibroma, pyogenic granuloma, peripheral giant cell granuloma, and fibrous histiocytoma) and malignant lesions (malignant fibrous histiocytoma and synovial sarcoma. Certain other differentials include rarer stromal sarcomas such as fibrosarcomas, mesenchymal chondrosarcomas, vascular leiomyomas, and juvenile hemangiomas). Fibrous histiocytoma shows a storiform or cartwheel pattern and a less prominent vascular network. Synovial sarcoma may show a biphasic cellular pattern and include fibrosarcoma-like areas. Mesenchymal chondrosarcoma cells are smaller than those of a hemangiopericytoma, and well-defined islands of cartilage are present.[2],[10] Reticulin staining can be helpful in such cases to arrive at a definitive diagnosis of hemangiopericytomas as it is a special stain that demonstrates lesional vessels lined by a single layer of endothelial cells with the pericytes covering the basement membrane of blood vessel. Histopathology of hemangiopericytomas plays a crucial role as the treatment of this lesion is dependent on the amount of cellular atypia and mitotic activity present in the lesion. The more bland lesions with minimal mitotic activity are treated by a wide base excision while the more active and dysplastic lesions are treated by radical excisions with or without adjunct radio and chemotherapies.

Surgery forms the mainstay of treatment of hemangiopericytomas; the indications for adjunctive treatment are unknown and controversial. In the largest most recent head and neck case series, 4 out of 12 patients received postoperative external beam radiation to a median dose of 60 Gy for positive surgical margins, high-grade histology, or recurrent lesions. While there are several reports of adjunctive radiation and chemotherapy in a few case series, there are no large-scale studies looking at outcomes of postoperative adjunctive treatment.

 > Conclusion Top

Hemangiopericytoma is an extremely rare vascular neoplasm with a propensity for local recurrence, unpredictable behavior, and the potential for distant metastasis. Due to the paucity of cases reported in the literature, the clinical outcome, prognosis, and indications for postoperative adjunctive treatment are unknown. Otolaryngologists need to be aware of this rare tumor that can be treated successfully with surgical resection. Close long-term follow-up is needed since a recurrence can present many years after initial treatment.

Learning points

  1. This is one of the rarest malignancy occurring in pediatric age group and so any mass presenting in supraglottic region, this differential must be borne in mind
  2. As this malignancy is rare, very few case reports are available to guide the treatment such case reports add to the knowledge of various treatment strategies to be used in such a rare malignancy.

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Conflicts of interest

There are no conflicts of interest.

 > References Top

Markku M, Sharon WW. Soft tissue tumours. In: Ivan D, James L, editors. Anderson's Pathology. Missouri, USA: Mosby; 1996. p. 2501-2.  Back to cited text no. 1
Stout AP, Murray MR. Hemangiopericytoma: A vascular tumor featuring Zimmermann's pericytes. Ann Surg 1942;116:26-33.  Back to cited text no. 2
Carew JF, Singh B, Kraus DH. Hemangiopericytoma of the head and neck. Laryngoscope 1999;109:1409-11.  Back to cited text no. 3
Sleepski M, Piotrowiak I, Wlodarezyk Z. Local recurrence and distance metastases 18 years after resection of the greater omentum haemangiopericytoma. World J Surg Oncol 2007;63:1477-8.  Back to cited text no. 4
Harirchian S, Mirani NM, Baredes S. Hemangiopericytoma of the larynx. Auris Nasus Larynx 2013;40:98-102.  Back to cited text no. 5
Billings KR, Fu YS, Calcaterra TC, Sercarz JA. Hemangiopericytoma of the head and neck. Am J Otolaryngol 2000;21:238-43.  Back to cited text no. 6
Koch M, Nielsen GP, Yoon SS. Malignant tumors of blood vessels: Angiosarcomas, hemangioendotheliomas, and hemangioperictyomas. J Surg Oncol 2008;97:321-9.  Back to cited text no. 7
Mena H, Ribas JL, Pezeshkpour GH, Cowan DN, Parisi JE. Hemangiopericytoma of the central nervous system: A review of 94 cases. Hum Pathol 1991;22:84-91.  Back to cited text no. 8
Zweckberger K, Jung CS, Mueller W, Unterberg AW, Schick U. Hemangiopericytomas grade II are not benign tumors. Acta Neurochir (Wien) 2011;153:385-94.  Back to cited text no. 9
Tang JS, Gold RH, Mirra JM, Eckardt J. Hemangiopericytoma of bone. Cancer 1988;62:848-59.  Back to cited text no. 10


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


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