Post-transplantation malignancies are well known complications after liver transplantation. Certain malignancies are more common in pediatric recipients than adults. Inflammatory myofibroblastic tumors (IMTs) are reactive neoplasms with miniscule malignant potential. IMTs are more common after hematopoietic stem cell transplantation. However, there is 1 case reported in the literature after deceased donor liver transplantation. The authors describe a case of IMT after living donor liver transplantation. The patient was a 1-year-old girl who underwent living donor liver transplantation (LDLT) for decompensated cirrhosis secondary to extra hepatic biliary atresia. Six months post LDLT routine ultrasonography revealed multiple solid abdominal masses. Repeated biopsies were inconclusive. Hence surgical excision was carried out. Histopathological examination revealed IMT. Immunohistochemistry was positive for anaplastic-lymphoma kinase activity. Ceritinib, a tyrosine kinase inhibitor, was used as adjuvant chemotherapy for 1 year. At 1.5 years (at the time of writing this paper) of follow-up, the child was disease free on imaging (whole body positron emission tomography-computed tomography). This will be the first case of IMT after LDLT to be reported in the literature.
Post-transplantation malignancies are well known complication of chronic immunosuppression. Certain malignancies are more common in pediatric recipients than in adults. Literature review revealed that inflammatory myofibroblastic tumors (IMTs) generally occur 3 to 6 months post-transplantation and are more common after hematopoietic stem cell transplantation (HSCT). Epstein-Barr virus has been held responsible in some reported cases. Lungs are the commonest site however they can present anywhere in the body. We describe a case of 6 months old female child who was suffering from end stage liver disease secondary to extra-hepatic biliary atresia (EHBA). She underwent living donor liver transplantation (LDLT). Six months post liver transplantation she was found to have intra-abdominal tumors on routine ultrasonography (USG). She underwent surgical excision of peritoneal neoplasms. Histopathological examination (HPE) confirmed IMT with anaplastic lymphoma kinase activity. She received ceritinibas chemotherapy for 1 year. At 1.5 years of follow-up, the child is recurrence free.
Our patient was a 1-year-old female child from Pakistan. She had decompensated chronic liver disease secondary to EHBA. She underwent primary LDLT at the age of 6 months at our center. Her mother was the donor. She had uneventful post transplantation recovery. At 3 months post liver transplantation (LT), routine USG revealed solid masses in the pelvis and in the sub hepatic region. Trucut biopsy of both the lesions revealed mesenchymal neoplasm. There was no evidence of malignancy. There was gradual increase in size of tumor on follow-up scans over the next 3 months
Clinical pictures of the case report. (A) Cross sectional image showing the tumor along the cut surface of the graft liver; (B) coronal section of the PET-CT image showing PET avid lesions; (C) intra-operative picture showing one tumor near the cut surface of graft liver; (D) intra-operative picture showing the tumor masses along with the transplanted liver. PET: positron emission tomography; CT: computed tomography
Various magnifications (A: ×4; B: ×10; C: ×20; D: ×40) of haematoxylin and eosin staining showing liver parenchyma along with few malignant spindle cells
IHC panel. (A) Negative result of IHC for SMA; (B) positive result of IHC for ALK-1l; (C) IHC for C-Kit shows tumor cells with mitotic index of less than 5%; (D) IHC for MIB index. IHC: immunohistochemistry; ALK: anaplastic lymphoma kinase; SMA: smooth muscle actin
The risk of
IMTs are one of the rare groups of post-transplant malignancies. More than 200 cases have been described so far. Majority of them are after HSCT.[
In our case, multiple preoperative biopsies were not confirmatory and led to delay in treatment. Final excision biopsy was subjected to extensive evaluation with IHC panel which included c-KIT, ALK, CD117, CD 45, CK-7, neuron specific enolase, chromogranin, alpha fetal protein, desmin, actin. The tumor in this case was negative for other IHC markers including those specific for hepatocellular carcinoma, cholangiocarcinoma, neuroendocrine tumors, gastrointestinal stromal tumors and lymphomas. However, the tumor had classic characteristics of IMT along with positive IHC for anti-lymphoma kinase. Complete tumor excision is curative in most cases.[
In conclusion: (1) although IMTs are rare, it is a serious complication after liver transplantation; (2) only one case has been reported in the literature so far after liver transplantation that too after DDLT; (3) in this case report we have described the first case of IMT after LDLT; (4) definitive histologic evidence is essential in their diagnosis and differentiation from other malignant tumors; and (5) IHC based targeted therapy may be helpful as adjuvant.
Doing surgery and writing of this manuscript: N. Selvakumar
Preparing the slides for histopathology: P. Saboti
Reporting of HPE: S. Kaul
Chief surgeon in charge of the case: S. Gupta
None.
There are no conflicts of interest.
Patient consent was received.
Since it is not a study and just a report, ethical approval is not needed as per our hospital policies.