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Year : 2019  |  Volume : 62  |  Issue : 2  |  Page : 303-305
Posttransplant epithelioid inflammatory myofibroblastic sarcoma: A case report

Department of Histopathology, Apollo Hospital, New Delhi, India

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Date of Web Publication10-Apr-2019


Epithelioid inflammatory myofibroblastic sarcoma (EIMS) is a rare entity and a novel variant of inflammatory myofibroblastic tumor (IMT), usually seen in children and nonsmoking young adults. Their occurrence in a posttransplant setting is still rare. These tumors are characterized by prominent epithelioid morphology, large histiocytoid “Reed Sternberg”-like cell, unique pattern of ALK immuno-reactivity, and aggressive clinical behavior. Their etiology and metastatic potential is controversial. In a post-transplant setting, many factors such as trauma, infections with EBV, HIV, Hepatitis C, mycobacteria, fungus, and chemotherapy-induced immunosuppression have been implicated in their etiology. We present the case of a 2-year-old female child who developed multiple omental and mesenteric tumor nodules, 8 months post liver transplant for progressive familial intrahepatic cholestasis (PFIC). Following a histopathological diagnosis of “mesenchymal neoplasm of possible malignant nature” on a trucut biopsy and frozen section, tumor debulking was performed. A final histological diagnosis of EMIS was made on the completely resected tumor. The patient remains in remission nearly 7 months after presentation, without any follow-up systemic chemotherapy. IMT after a solid organ transplant is rare, only 5 cases have been reported in the literature until now. Similar phenomenon has also been noted with hematopoietic stem cell transplant. However, to our knowledge, this case of EMIS in a post liver transplant patient is first of its kind.

Keywords: EIMS in post solid organ transplant, epithelioid inflammatory myofibroblastic sarcoma in liver transplanted child, inflammatory myofibroblastic tumor (IMT) in liver, progressive familial intrahepatic cholestasis (PFIC)

How to cite this article:
Garg R, Kaul S, Arora D, Kashyap V. Posttransplant epithelioid inflammatory myofibroblastic sarcoma: A case report. Indian J Pathol Microbiol 2019;62:303-5

How to cite this URL:
Garg R, Kaul S, Arora D, Kashyap V. Posttransplant epithelioid inflammatory myofibroblastic sarcoma: A case report. Indian J Pathol Microbiol [serial online] 2019 [cited 2022 Oct 7];62:303-5. Available from:

   Introduction Top

Inflammatory myofibroblastic tumor (IMT) is a mesenchymal neoplasm of intermediate biological potential which rarely recurs or metastasize.[1] They present as solitary or multiple mass lesions in the abdominopelvic region, lung, mediastinum, and retroperitoneum of children and young adults.[1] Only 8 cases of post hematopoietic stem cell transplantation (HSCT) IMTs and even fewer in post solid organ transplant patients have been reported till date.[2],[3],[4]

In 2011, Mariño-Enríquez et al.[2] described a novel variant of IMT – epithelioid inflammatory myofibroblastic sarcoma (EIMS). They are characterized by an epithelioid morphology and prominent neutrophilic inflammatory infiltrate. Clinically, they are more aggressive tumors with a shorter disease-free survival.[3] Approximately 50% of IMTs aberrantly express clonal rearrangements of ALK gene located on chromosome 2p235.[5] Only 20 cases of EIMS have been reported thus far but none in a posttransplant setting.[6] To the our knowledge, this is the first case of IMT possibly of epithelioid sarcomatous type in a liver transplanted child.

   Case Report Top

An 18-month-old female child received a liver transplant in October 2015 for progressive familial intrahepatic cholestasis and was discharged after an uneventful postoperative period in stable condition. No intra-abdominal mass lesions were detected on radiology before and soon after the transplant. In May 2016, she presented with fever and distended abdomen. Computed tomography (CT) abdomen and positron emission tomography (PET) scan showed fludeoxyglucose (FDG)-avid multiple lesions in omentum and subdiaphragmatic area and attached to the serosa of small bowel. Their trucut biopsies revealed a mesenchymal neoplasm, positive for vimentin and SMA [Figure 1]. All other markers, such as caldesmon, C-kit, CD 34, S100, and CD 31, were negative. Because the MIB-1 index was 15–20%, the suspicion of malignancy was high. A more representative sample was required for final tumor typing. The patient was operated upon, with a plan to resect all the tumors.
Figure 1: Mesenchymal neoplasm, 10X, 20X, positive for Vimentin. Ki-67 index 15-20%

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Two large masses measuring 16 cm and 8 cm in their maximum dimensions and two smaller nodules (0.5 cm, 1 cm) attached to the bowel were excised. Their external surface was uniform smooth and gray white. The cut section was vaguely lobulated, gray white, and myxoid. Tumor masses from omentum and small bowel showed large areas of necrosis and hemorrhage. Histomorphology reveals plump epithelioid to spindle-shaped cells with indistinct pale, eosinophilic cytoplasm. Pleomorphic nuclei show vesicular chromatin and distinct nucleoli. A rich network of thin-walled delicate capillaries with lympho-plasmacytic infiltrate and few neutrophils. Mitotic figures are rare [Figure 2].
Figure 2: Plump epitheloid to spindle shaped cells in a background of rich network of thin walled delicate capillaries(2A, 2B) with lymphoplasmacytic infiltrate and few neutrophills. Pleomorphic nuclei with distinct nucleoli and rare Mitosis (2C, 2D)

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The morphology and initial immuno-profile suggested differential diagnoses of myxofibroblastic sarcoma, low-grade fibromyxoid sarcoma, and IMT. In view of positivity for vimentin, desmin, SMA, ALK-1, and Ki-67 index of 15–20% [Figure 3], a diagnosis of EBV-negative IMT, possibly of epithelioid type was established.
Figure 3: Tumor cells are positive for Vimentin, Desmin, SMA and ALK-1. EBV is negative, MIB-1 index 15-20%

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   Discussion Top

De novo neoplasms account for almost 30% of deaths 10 years after liver transplantation and are the most common causes of mortality in patients surviving at least 1 year after transplant. The risk of malignancy is 2–4 times higher in transplant recipients than in an age- and sex-matched population.[7] Skin cancers, lymphomas, and Kaposi sarcomas occur most frequently which can be contributed to chronic immunosuppression after solid organ transplantation and EBV infection.[6],[7] IMT with prominent epithelioid cytomorphology is very rare. In 4 out of 73 cases studied, Cook et al. found round cell transformation and large polygonal cells with a large nuclei and prominent nucleoli in a loose, pale staining background.[8] One of these, 4 cases showed distinctive nuclear membrane staining pattern of ALK. In 2010, Butrynski et al. reported sustained partial response to the ALK inhibitor, Crizotinib, in a patient with ALK-translocated IMT with epithelioid cytomorphology.[9] The clinical, morphological, and immunohistochemical features of 20 cases of reported EIMS are summarized in [Table 1]. All of these showed increased expression of ALK protein resulting from RANBP2-ALK fusion. Accordingly, we infer that RANPB2-ALK fusion gene might be a potential molecular mechanism for the rapid growth and recurrence of EIMS.
Table 1: Clinical, morphologic and immune-histochemical summary of “Epithelioid inflammatory myofibroblastic sarcoma”

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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

Fletcher CDM. Soft tisuue tumors. In: Bridge JA, Hogendorn PCW, Mertens F, editors. Diagnostic Histopathology of tumors. 4th ed. Lyon: IARC Press; 2013. p. 1823-4.  Back to cited text no. 1
Asli T, Gulistan B. Inflammatory pseudotumors after stem cell transplantation Hematol Rep 2015;7:5848.  Back to cited text no. 2
Tepeoǧlu M, Atılgan AO, Ozdemir B, Haberal M. Synchronous Post transplant Lymphoproliferative Disorder and Inflammatory Myofibroblastic Tumor of the Lung in a 2-Year-Old Liver Transplanted Boy: A Case Report. Exp Clin Transplant 2015;1:92-5.  Back to cited text no. 3
Hammas N, Chbani L, Rami M, Boubbou M, Benmiloud S, Bouabdellah Y, et al. A rare tumor of the lung: Inflammatory myofibroblastic tumor. Diagn Pathol 2012;7:83.  Back to cited text no. 4
Mariño-Enríquez A, Wang WL, Roy A, Lopez-Terrada D, Lazar AJ, Fletcher CD, et al. Epithelioid inflammatory myofibroblastic sarcoma: An aggressive intra-abdominal variant of inflammatory myofibroblastic tumor with nuclear membrane or perinuclear ALK. Am J Surg Pathol 2011;35:135-44.  Back to cited text no. 5
Rosenbaum L, Fekrazad MH, Rabinowitz I, Vasef MA. Epstein–Barr virus-associated inflammatory pseudotumor of the spleen: Report of two cases and review of the literature. J Hematop 2009;2:127-31.  Back to cited text no. 6
Burra P, Castro KIR. Neoplastic disease after liver transplantation: Focus on de novo neoplasms. World J Gastroenterol 2015;21;8753-68.  Back to cited text no. 7
Cook JR, Dehner LP, Collins MH, Ma Z, Morris SW, Coffin CM. Anaplastic lymphoma kinase (ALK) expression in the inflammatory myofibroblastic tumor: A comparative immunohistochemical study. Am J Surg Pathol 2001;25:1364-71.  Back to cited text no. 8
Patel AS, Murphy KM, Hawkins AL, Cohen JS, Long PP, Perlman EJ, et al. RANBP2 and CLTC are involved in ALK rearrangements in inflammatory myofibroblastic tumors. Cancer Genet Cytogenet 2007;176:107-14.  Back to cited text no. 9

Correspondence Address:
Ritu Garg
Department of Histopathology, Apollo Hospital, New Delhi - 110 025
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/IJPM.IJPM_284_17

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  [Figure 1], [Figure 2], [Figure 3]

  [Table 1]

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