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Year : 2018  |  Volume : 7  |  Issue : 1  |  Page : 70-73

Undifferentiated pleomorphic sarcoma of maxilla presenting like a periapical pathology – A case report

1 Department of Oral and Maxillofacial Surgery, SRM Kattankulathur Dental College, Potheri, Kanchipuram, Tamil Nadu, India
2 Department of Oral Pathology, SRM Kattankulathur Dental College, Potheri, Kanchipuram, Tamil Nadu, India

Date of Web Publication22-Mar-2018

Correspondence Address:
Dr. K T Magesh
Department of Oral Pathology, SRM Kattankulathur Dental College, Potheri, Kanchipuram - 603 203, Tamil Nadu
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Undifferentiated pleomorphic sarcoma (UPS) previously described as malignant fibrous histiocytoma (MFH) is a common soft tissue sarcoma, but relatively rare in oral cavity. In this article we report a case of 23-year-old male patient diagnosed UPS in left side of maxilla anterior region which had a deceptive clinical and orthopantomogram findings. Patient had diffuse swelling left upper side of the face with periapical radiolucency involving 21, 22, 23 regions and decreased response to electric pulp testing in those teeth. Patient underwent surgical removal of the entire lesion with wide marginal clearance followed by radiation and chemotherapy. This case report highlights the importance of tissues to be sent for histopathological examination for definitive diagnosis.

Keywords: Malignant fibrous histiocytoma, periapical pathology, undifferentiated pleomorphic sarcoma

How to cite this article:
Vivek N, Magesh K T, Sivachandran A, Kumar M S. Undifferentiated pleomorphic sarcoma of maxilla presenting like a periapical pathology – A case report. J NTR Univ Health Sci 2018;7:70-3

How to cite this URL:
Vivek N, Magesh K T, Sivachandran A, Kumar M S. Undifferentiated pleomorphic sarcoma of maxilla presenting like a periapical pathology – A case report. J NTR Univ Health Sci [serial online] 2018 [cited 2020 Apr 4];7:70-3. Available from: http://www.jdrntruhs.org/text.asp?2018/7/1/70/228147

  Introduction Top

Sarcomas arising in the head and neck region have a lower incidence than epithelial tumors accounting up to 4–10%, the most frequent being the neck and parotid gland.[1] Of these, about only 0.1% accounts for the undifferentiated pleomorphic sarcomas (UPS) of the oral cavity.[2] UPS was first described by Ozello et al. in 1963 as MFH and Feldman and Norman (1972) described it as primary malignant tumor of bone. It commonly involves the extremities and retroperitoneum.[3]

In the head and neck region the nasal cavity and the paranasal sinuses are the commonest sites. Unlike carcinomas, in sarcomas there are numerous subtypes without known tissue or cell of origin.[3] UPS essentially represents a diagnosis of exclusion.[1] In this article, we describe a case which presented similar to periapical pathology, but showed no specific line of differentiation on histopathological examination.

  Case Report Top

A 23-year-old male patient reported to the SRM Kattankulathur Dental College with a swelling in the left side of the face [Figure 1] for 2 months duration. The swelling increased in size gradually and was tender on palpation. Intraorally the swelling was seen in relation to 21, 22, 23 region obliterating the buccal sulcus [Figure 2]. Patient gave a history of trauma 6 months before in the left upper anterior region. Orthopantomogram revealed a radiolucency in the periapical region of the 21, 22, 23 teeth [Figure 3] with decreased response to electric pulp testing. No mobility of tooth was noticed and the patient had no other significant medical history.
Figure 1: Extraoral photograph reveals diffuse swelling on the left side of the face in the maxillary anterior region

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Figure 2: Intraoral examination shows swelling at the junction of gingival and labial sulcus in the maxillary anterior region

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Figure 3: Orthopantomogram reveals ill-defined radiolucency in relation to 21 to 23 regions with resorption of roots

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Considering the X-ray findings and the history of trauma, periapical pathology was suspected. Since the size of the lesion was large, an incisional biopsy was performed.

Computerized tomography (CT) scan was performed to know the extent of the lesion and it revealed a huge tumor mass in the left side maxilla extending into the maxillary sinus, nasal cavity and involving the hardpalate [Figure 4]. However, whole body CT scan did not reveal any metastasis.
Figure 4: Computed tomography reveals a soft tissue mass in relation to the left maxilla involving the floor of the nasal cavity

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The histopathological evaluation, the microscopic examination revealed biphasic pattern of anaplastic tumor cells which contains both spindle shaped cells with vaguely storiform growth pattern [Figure 5] and rounded histiocytes-like cells suggesting aggressive spindle cell neoplasm.
Figure 5: Microscopic examination of H and E (10x) stained section shows spindle cells in herring bone pattern

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Partial maxillectomy was performed and the entire tumor mass was removed with wide surgical marginal clearance. Dental obturator was given to the patient to assist feeding.

Histopathological evaluation of the excised specimen revealed undifferentiated pleomorphic spindle shaped cells arranged in herringbone pattern and round histiocyte-like cells were also seen [Figure 6]. Immunohistochemistry was performed and it showed positivity for vimentin [Figure 7] and was negative for EMA, desmin, myogenin, SMA, CD34, and S-100. A diagnosis of UPS was given based on the histopathology and immunohistochemical profile and the patient was referred to the Cancer Institute for Chemotherapy and Radiation. All the margins of the excised specimen were free of tumors.
Figure 6: Microscopic examination of H and E (10x) stained section shows biphasic pattern of anaplastic tumor cells and contains both spindle-shaped and rounded histiocytic cells

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Figure 7: Immunohistochemical staining shows vimentin positivity for both spindle shaped and rounded histiocytic cells

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  Discussion Top

The term “malignant fibrous histiocytoma (MFH)” dates back to early 1960s. The next two decades confirmed and expanded the diagnostic entity of MFH.[4] Five histological subtypes have been described such as storiform/pleomorphic, myxoid, giant cell, inflammatory, and angiomatoid.[5] The term MFH was being overused and it had become nothing more than a diagnosis of exclusion.[6] WHO (2002) classified the sarcomas and revamped the term MFH to “UPS.”[4]

Squamous cell carcinoma and lymphoma account for the most common malignancy (80–90%) of head and neck region. Sarcoma including UPS has less than 10% of representation in head and neck region. Soft tissue UPS are common in lower extremities, head and neck region its occurrence is rare. In our case, involvement of maxilla shows its uniqueness and more over its aggressiveness than the rest of sites.[1]

Predisposing factors such as history of trauma, surgery, radiation, fractures, osteonecrosis, Paget's disease, non-ossifying fibroma, and fibrous dysplasia have been reported.[7] The history of trauma 6 months before was present in our case.

The majority of literature have reported with male predominance with male to female ratio of 2:1.[8] Our case was in concurrence with the sited reports.

Clinically, MFH usually presents as an enlarging, painless soft tissue mass that may be associated with nonspecific symptoms.[1],[5] Imaging studies were also nonspecific with orthopantomogram showing diffuse radiolucency in the region of 21,22,23 involving the roots. The radiolucency was similar to the radiolucency found in the periapical pathosis.

The definitive diagnosis was established by means of histopathological examination. Microscopically, the tissue specimen exhibits heterogeneity of cell pattern, pleomorphism, and aberrant giant cells with lack of particular line of cell differentiation. The exact histiogenesis of UPS remains mystery and leaving some gray areas. Initially, it was suggested that the histiocytes, which characterize the disease were acting as “facultative fibroblasts.” The new school of thought is undifferentiated mesenchymal cells are the source of differentiation of histiocytic and fibrocytic cell lineage. The differential diagnosis includes spindle cell carcinoma, carcinosarcoma, and fibrosarcoma.[9]

In order to differentiate it from other tumors, immunohistochemical profile was done and showed EMA, desmin, myogen, and SMA negativity ruling out the possibility of adenocarcinomas and sarcomas of muscle cells. Negative response to CD34 and S100 markers helps us to exclude the diagnosis from vascular and nerve tissue origin. However, it was positive for vimentin alone suggesting that the tumor cells originated from mesenchymal cells.[3]

The management of sarcomas includes surgery, followed by radiotherapy and chemotherapy.[1] In our case, surgical removal of the mass was done with partial maxillectomy. Obturator was given to restore functions of oral and nasal cavities. Patient was referred to Adyar Cancer Institute for adjuvant therapy of radiotherapy as well as chemotherapy. Metastases have been reported frequently in the lung. However, cervical metastases are rare. Prognosis depends on the size of the tumor and its malignant potential in terms of metastasis.[10] In our case, distant metastases was not evident initially. Subsequently, after 3 months of chemotherapy and radiotherapy, the patient developed difficulty in breathing. Investigations were performed to rule out any metastasis. CT scan revealed huge nodule involving the mediastinum and subsequently leading to death.

  Conclusion Top

UPS of oral cavity is a rare neoplasm of the oral cavity. It presents as a rapidly growing mass in the oral cavity associated with a local swelling and pain. Because of the nonspecific clinical and imaging features, histopathology becomes essential for its diagnosis by exclusion with immunohistochemical profile. The difficulty in management is that limited number of cases has been reported so far to harbor a definitive treatment plan and improve the prognosis of the patients.

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.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Sepulveda I, Compan A, Ulloa J, Mucientes P, Ascui R, Fredes F. Maxillary undifferentiated high-grade pleomorphic sarcoma: A case report and review of the literature. Case Rep Clin Pathol 2017;4:32-5.  Back to cited text no. 1
Mori Y, Motoi T, Ida K, Shibahara J, Saijo H, Susami T, et al. Primary undifferentiated high-grade pleomorphic sarcoma/malignant fibrous histiocytoma arising from the mandible. Oral Sci Int 2014;70-5.  Back to cited text no. 2
Kim CH, Jang JW, Kim MY, Kim YH, Kim HG, Kim JH. Undifferentiated pleomorphic sarcoma in mandible. Maxillofac Plast Reconstr Surg 2014;36:303-7.  Back to cited text no. 3
Matushansky I, Charytonowicz e, Mills J, Siddiqui S, Hricik T, Cardo CC. MFH classification: Differentiating undifferentiated pleomorphic sarcoma in the 21st century. Expert Rev Anticancer Ther 2009;9:1135-44.  Back to cited text no. 4
Lambade PN, Lambade D. Malignant fibrous histiocytoma: An Uncommon sarcoma with pathological fracture of mandible. J Maxillofac Oral Surg 2015;14:283-7.  Back to cited text no. 5
Enzinger FM. Malignant fibrous histiocytoma 20 years after stout. Ame J Surg Pathol 1986;10:43-53.  Back to cited text no. 6
Pobirci DD, Bogdan F, Pobirci O, Petcu CA, Rosca E. Study of malignant fibrous histiocytoma: Clinical, statistic and histopathological interrelation. Rom J Morphol Embryol 2011;52:385-8.  Back to cited text no. 7
Kasat VO, Saluja H, Rudagi BM, Kalburge JV, Sachdeva S. Malignant fibrous histiocytoma of maxillary alveolar ridge extending to the hard palate. J Cancer Res Ther 2014;10:422-4.  Back to cited text no. 8
Santoni T, Watanabe M, Kaneko T, Matsubayashi J, Nagao T, Chiba H. Radiation induced malignant fibrous histiocytoma of the maxilla. Odontology 2011;99:203-8.  Back to cited text no. 9
Sreenivasan BS, Sankar VV, Mathew DG, George M. Undifferentiated pleomorphic sarcoma presenting as a gingival mass: A rare case. J NTR Univ Health Sci 2015;4:130-33.  Back to cited text no. 10
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  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7]


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