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Peritoneal sarcomatosis due to undifferentiated pleomorphic sarcoma: A case report and review of the literature


1 Department of Pathology, Gazi Yasargil Research and Training Hospital, University of Health Sciences, Diyarbakir, Turkey
2 Department of Radiology, Gazi Yasargil Research and Training Hospital, University of Health Sciences, Diyarbakir, Turkey
3 Department of General Surgery and Surgical Oncology, Faculty of Medicine, Cukurova University, Adana, Turkey

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Date of Submission09-Mar-2021
Date of Decision29-Jun-2021
Date of Acceptance07-Sep-2021
Date of Web Publication27-Apr-2022
 

   Abstract 


Undifferentiated pleomorphic sarcoma (UPS), which was previously known as malignant fibrous histiocytoma (MFH), rarely presents in the abdomen, and sarcomatosis due to UPS has not yet been reported in the literature. Here, we present a 62-year-old man who had abdominal sarcomatosis due to UPS with a poor prognosis.

Keywords: Abdomen, malignant fibrous histiocytoma, peritoneal sarcomatosis, undifferentiated pleomorphic sarcoma


How to cite this URL:
Sogutcu N, Kavak S, Gumus S. Peritoneal sarcomatosis due to undifferentiated pleomorphic sarcoma: A case report and review of the literature. Indian J Pathol Microbiol [Epub ahead of print] [cited 2022 Aug 11]. Available from: https://www.ijpmonline.org/preprintarticle.asp?id=344186





   Introduction Top


Malignant fibrous histiocytoma (MFH), which was included in the undifferentiated pleomorphic sarcoma (UPS) group with the classification of soft tissue tumors by the World Health Organization in 2013, first defined as a storiform growth pattern histiocytic tumor by Kauffman in 1961.[1],[2] UPS, considered a type of malignant sarcoma, is usually seen in older patients.[3] It constitutes 28% of all soft tissue sarcomas.[4] The location of the tumor is in the lower (49%) and upper (19%) extremities, followed by 16% in the retroperitoneal and abdominal cavity and 9% in the trunk, respectively.[5]

This case report presents a new case of abdominal UPS with peritoneal metastasis, which is extremely rare and had a poor prognosis; it is characterized by complicated clinical and histopathological diagnoses.


   Case History Top


A 62-year-old male patient was admitted to the emergency room with severe abdominal pain in February 2018. He had weakness and general condition disorder in his medical history and weight loss over the past few months. Physical examination revealed abdominal swelling. In the laboratory tests, no significant feature was found except leukocytosis and high C-Reactive protein (CRP). Air-fluid levels were found on anterior-posterior abdominal X-ray radiography. An intravenous contrast-enhanced abdominal computed tomography revealed a mass in the pelvic region with lobulated contours, irregularly circumscribed with the bowel loops' localization, accompanied by multiple mesenteric implants located in all quadrants of the abdomen [Figure 1]. An emergency laparotomy was performed, and malignant implants with a bleeding tendency were observed in all small intestine segments and colon. The small intestine segment from 30 cm distal to the trietz to 30 cm proximal to the terminal ileum and the sigmoid colon approximately 15 cm in length and omentum were resected.
Figure 1: Contrast-enhanced abdominal CT axial sections. (a) Significant distension (arrows) in the small intestine segments and solid mass lesions (asterisks) from the wall and protruding out of the lumen in the small intestine segments are shown. (b) Solid mass lesions enhancing in small intestine segments and descending colon level (asterisks) are seen. (c) On the axial section passing through the pelvic level, a mass lesion filling the pelvis and protruding into the lumen associated with the ileal loop is observed (asterisk)

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A tumor with a size of 9 × 8 × 6 cm in the small intestine and 5.5 × 2.5 × 1.5 cm in the sigmoid colon was observed macroscopically in the pathological examination. There were also many tumoral implants, the largest of which was 7 × 2.5 × 2.5 cm in the omentum. The cross-sectional tumor surface was grayish-white in color and solid character with focal hemorrhagic areas. In the microscopic examination, an invasive tumor was observed in all small intestine layers from mucosa to serosa [Figure 2]. Cancer in the colon surface was only invasive to serosa and muscularis propria. In the immunohistochemical study, tumor cells were diffuse positive with vimentin and CD68 [Figure 3], but PanCK, S100, SMA, desmin, CD34, HMB45, EMA, and CEA were negative [Figure 4]. The Ki-67 proliferation index was about 60% [Figure 5]. The patient was diagnosed with high-grade UPS with the present pathological findings.
Figure 2: Microscopic examination with hematoxylin-eosin staining. (a) Tumoral cells are showing a storiform growth pattern in the mucosa and submucosa (HE × 100). (b) Pleomorphic and bizarre tumor cells are with foamy cytoplasm and prominent atypia in the collagen stroma. Among the tumor cells, inflammatory cells and mitotic figures stand out. (HE × 200)

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Figure 3: Diffuse and strong positive staining of tumor cells with vimentin and CD68 (×100). (a) Positive staining with vimentin. (b) Positive staining with CD68

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Figure 4: Negative staining with CD117, CD45, S100, PanCK, HMB45, and desmin in tumor cells (×100). (a) Negative staining with CD117. (b) Negative staining with CD45. (c) Negative staining with S100. (d) Negative staining with PanCK. (e) Negative staining with HMB45. (f) Negative staining with desmin

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Figure 5: High staining with Ki67 in tumor cells (×100)

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It was decided to give adjuvant radiotherapy to the patient evaluated in the multidisciplinary tumor council after surgery. The treatment could not be continued due to gastrointestinal bleeding and general condition disorder that developed after radiotherapy. The patient died in April 2018.


   Discussion Top


Patients with abdominal UPS usually present to the hospital for fever, abdominal pain, palpable mass, diarrhea, blood in the stool, and appetite loss. Nonspecific symptoms may develop in patients due to weight loss and increased intra-abdominal pressure.[5] UPS's preoperative diagnosis is challenging, as there are no specific clinical and radiological findings, and no significant elevation in tumor markers is detected. Therefore, when patients are diagnosed, the tumor size is usually large.[6] In addition to clinical, radiological, and laboratory findings, an accurate evaluation and immunohistochemical study by histopathological criteria are required to diagnose UPS.[3]

UPS is divided into four histological types: storiform-pleomorphic, myxoid, giant cell, and inflammatory.[3] Our cases had histomorphology consisting of spindle-shaped and large histiocyte-like cells with prominent nucleoli arranged in a storiform pattern. Distinct pleomorphic cells were scattered in both our patients.

It has been reported immunohistochemically that UPS usually expresses CD68, vimentin, actin, α-1-antitrypsin, and frequently laminin mRNA.[7] However, neither a reproducible immunophenotype nor any protein expression in the tumor allows for a more specific subclassification.[8]

Intra-abdominal UPS probably has a worse prognosis than those located in the extremity due to late diagnosis. Although the number of reported cases is limited, many investigators consider intra-abdominal UPS as a rare but aggressive tumor.[6] Tumor size is a significant prognostic factor along with grade.[8] Wide surgical resection with negative tumor margins is the preferred primary treatment modality.[8] Metastases occur in the lung (82%), lymph nodes (32%), then bone and liver, respectively.[5] Recent studies suggest that some indicators such as phosphorylation of signal transducer and activator of transcription protein 3 (STAT3), 3'-untranslated region of suppressor of cytokine signaling 3 (SOCS3), MET, and KIT proteins overexpression may be useful prognostic factors for UPS.[9],[10] Another important criterion in the UPS prognosis is the ratio of the myxoid component.[3] The metastatic tendency of myxoid tumors was lower. Therefore, patients with myxoid tumors may not need systemic therapy, whereas patients with nonmyxoid tumors larger than 5 cm are at risk of developing a local recurrence or distance metastases.[3]

The use of adjuvant radiotherapy in treating patients with intraabdominal UPS is controversial, and it is not applied routinely. However, neoadjuvant radiotherapy can be considered successful radical resection in advanced stage retroperitoneal tumors.[3] Adjuvant radiotherapy was given to our patient after evaluating the multidisciplinary tumor council. The treatment was discontinued due to gastrointestinal bleeding and general condition disorder in the patient, and he died in the 4th month of follow-up.

To conclude, UPS is an uncommon tumor and rarely presents with peritoneal sarcomatosis. Diligent evaluation of histopathological features is critical at diagnosis. Also, there are no standard recommendations for therapeutic strategy given these tumors' rarity, and the prognosis is poor.

Declaration of patient consent

The consent form was obtained from the patient at the first admission to the hospital for future studies. The authors certify that they have obtained all appropriate patient consent forms. In the form the patient has given his/her/their consent for his images and other clinical information to be reported in the journal. The patient understand that his name and initials will not be published and due efforts will be made to conceal his identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Kauffman SL, Stout AP. Histiocytic tumors (fibrous xanthoma and histiocytoma) in children. Cancer 1961;14:469-82.  Back to cited text no. 1
    
2.
Jo VY, Fletcher CD. WHO classification of soft tissue tumors: An update based on the 2013 (4th) edition. Pathology 2014;46:95-104.  Back to cited text no. 2
    
3.
Nascimento AF, Raut CP. Diagnosis and management of pleomorphic sarcomas (so-called “MFH”) in adults. J Surg Oncol 2008;97:330-9.  Back to cited text no. 3
    
4.
Cormier JN, Pollock RE. Soft tissue sarcomas. CA Cancer J Clin 2004;54:94-109.  Back to cited text no. 4
    
5.
Weiss SW, Enzinger FM. Malignant fibrous histiocytoma: An analysis of 200 cases. Cancer 1978;41:2250-66.  Back to cited text no. 5
    
6.
Karki B, Xu YK, Wu YK, Zhang WW. Primary malignant fibrous histiocytoma of the abdominal cavity: CT findings and pathological correlation. World J Radiol 2012;4:151-8.  Back to cited text no. 6
    
7.
Ji W, Zhong M, You Y, Hu KE, Wu B. Primary malignant fibrous histiocytoma of the colon: A case report and review of the literature. Mol Clin Oncol 2016;4:1006-8.  Back to cited text no. 7
    
8.
Fletcher CD, Bridge JA, Hogendoorn PC, Mertens F, editors. WHO Classification of Tumors of Soft Tissue and Bones. Lyon, France: IARC; 2013. p. 318-20.  Back to cited text no. 8
    
9.
Pazzaglia L, Novello C, Conti A, Pollino S, Picci P, Benassi MS. miR-152 down-regulation is associated with MET up-regulation in leiomyosarcoma and undifferentiated pleomorphic sarcoma. Cell Oncol (Dordr) 2017;40:77-88.  Back to cited text no. 9
    
10.
Bekki H, Kohashi K, Yamada Y, Iura K, Ishii T, Maekawa A, et al. Phosphorylation of STAT3 in undifferentiated pleomorphic sarcoma is correlated with a favorable prognosis. Pathobiology 2017;84:161-9.  Back to cited text no. 10
    

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Correspondence Address:
Serdar Gumus,
Department of General Surgery and Surgical Oncology, Cukurova University, Saricam, Adana - 01330
Turkey
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijpm.ijpm_267_21



    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]



 

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