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F. Azam, M. Chatterjee, Sheila Kelly, M. Pinto, A. Aurangabadkar, M. Latif, E. Marshall (2014)
Multifocal calcifying fibrous tumor at six sites in one patient: a case reportWorld Journal of Surgical Oncology, 12
(2016)
Calcifying fibrous tumor. Med (United States) 2016;95:e3690
Jen-Chieh Lee, H. Lien, C. Hsiao (2007)
Coexisting sclerosing angiomatoid nodular transformation of the spleen with multiple calcifying fibrous pseudotumors in a patient.Journal of the Formosan Medical Association = Taiwan yi zhi, 106 3
J. Dorpe, N. Ectors, Karel Geboes, André D'Hoore, Raphael Sciot (1999)
Is calcifying fibrous pseudotumor a late sclerosing stage of inflammatory myofibroblastic tumor?The American journal of surgical pathology, 23 3
N. Rosenthal, F. Abdul-Karim (1988)
Childhood fibrous tumor with psammoma bodies. Clinicopathologic features in two cases.Archives of pathology & laboratory medicine, 112 8
J. Fetsch, Elizabeth Montgomery, J. Meis (1993)
Calcifying Fibrous PseudotumorThe American Journal of Surgical Pathology, 17
T. Kuo, Tse-Ching Chen, Li‐yu Lee (2009)
Sclerosing angiomatoid nodular transformation of the spleen (SANT): Clinicopathological study of 10 cases with or without abdominal disseminated calcifying fibrous tumors, and the presence of a significant number of IgG4+ plasma cellsPathology International, 59
(2002)
Calcifying fibrous ?pseudotumor?: Clinicopathologic study of 15 cases and analysis of its relationship to inflammatory myofibroblastic tumorInt J Surg Pathol, 10
(2016)
Calcifying fibrous tumorMed (United States), 95
Hiroyuki Yanai (2020)
Calcifying Fibrous TumorDefinitions
A. Nascimento, R. Ruiz, J. Hornick, C. Fletcher (2002)
Calcifying Fibrous ‘Pseudotumor’International Journal of Surgical Pathology, 10
Karl Chen (2003)
Familial peritoneal multifocal calcifying fibrous tumor.American journal of clinical pathology, 119 6
C. Fletcher (2013)
WHO classification of tumours of soft tissue and bone
Calcifying fibrous tumour (CFT) is a rare benign tumour with non-specific anatomical distribution. We describe a case of a patient who presented with chronic generalised fatigue secondary to anaemia. Her symptoms did not improve while being on oral iron replacement therapy. Further endoscopic investigations were unremarkable. She had a computed tomography scan showing masses in the right pleural base and in the spleen. She then underwent splenic biopsy that only showed inflammatory changes. As her symptoms persisted, she was worked up for elective laparoscopic splenectomy during which she was found to have multiple peritoneal deposits. Biopsies were taken and the splenectomy was abandoned. The biopsies eventually showed changes consistent with CFT. This was conclusive for diagnosis of multifocal CFT. was initially found to have microcytic anaemia with low ferritin INTRODUCTION level which did not improve despite oral iron replacement. Both Calcifying fibrous tumour (CFT) was initially described in child- oesophago-gastro-duodenoscopy and colonoscopy were unre- markable. A subsequent computed tomography (CT) scan of the hood cases. Its aetiology remains unclear, and few postulations have been made. The symptoms are often non-specific. Owing abdomen and pelvis showed a splenic mass with a central area of calcification and a calcified 35 mm pleural-based nodule at to its potential to be multifocal, the diagnosis can be clinically challenging. However, CFT has a specific histological finding that the right costophrenic angle. The latter was discussed in the lung multi-disciplinary team becomes the gold standard to confirm the diagnosis. Further studies are needed be to clarify the pathophysiology of this rare (MDT) meeting. They concluded that the pleural mass was most likely due to post-inflammatory changes. In absence of disease. concerning features, further monitoring or intervention was deemed unnecessary. The splenic mass was then discussed in CASE REPORT the hepatobiliary MDT meeting. They recommended obtaining A 57-year-old female was referred from primary care with a tissue biopsy to aid diagnosis. However, it only showed fibrotic 3-year history of generalised fatigue. She has background of tissue with possible granulomas. The indeterminate histology ichthyosis, lichen sclerosis, asthma, depression and chronic her- prompted a repeat CT scan and further magnetic resonance pes simplex virus infection. She had hysterectomy due to abnor- imaging (MRI) to assess the mass further and exclude the mal smear cells, which was then proven benign on histology. She possibility of lymphoma. These scans showed stable appearance Received: November 7, 2020. Accepted: December 12, 2020 Published by Oxford University Press and JSCR Publishing Ltd. © The Author(s) 2021. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted reuse, distribution, and reproduction in any medium, provided the original work is properly cited. 1 Downloaded from https://academic.oup.com/jscr/article/2021/01/rjaa573/6102033 by DeepDyve user on 26 January 2021 2 N.N. Abd Kahar and A. Al-Mukhtar with yearly CT scan to monitor the lesions and to continue with iron supplementation. DISCUSSION CFT was initially reported by Rosenthal and Abdul-Karim in 1988 as ‘childhood tumour with psammoma bodies’ in two childhood cases [1]. Fetsch et al.[2] then described 10 similar cases in individuals with age range of 1–33 years, and the term ‘calcifying fibrous pseudotumour’ was introduced. The term ‘pseudotu- mour’ was used at that time as the underlying process was most likely inflammatory [2]. World Health Organisation recognized its potential for local recurrence [3] and renamed it as ‘calcifying fibrous tumour’ [4]. CFT can occur as solitary or multiple lesions. A review by Chorti et al.[5] looked into 161 cases reported based on the anatomical location. The most common ones include the stom- ach, small intestine, pleura, oesophagus, neck, mesentery, medi- Figure 1: CT image of a splenic mass astinum and peritoneum. Due to this wide distribution, patients present with non-specific symptoms such as anorexia, weight loss, generalised fatigue, dyspnoea and progressive weakness. However, individuals can also present with specific symptoms relatedtoorgansinvolved[5]. In our case, the patient initial presenting complaint was generalised fatigue and she was found to have anaemia. Subsequent imaging revealed pleural mass and splenic mass. Interestingly she did not complaint of any breathing problem or abdominal discomfort despite the above findings. The cause and pathogenesis of CFT is unclear but some hypotheses have been suggested in the past. Chen reported a case of two sisters with multifocal peritoneal CFT suggesting that there might be genetic susceptibility for CFT, although com- mon environmental factors cannot be discounted [6]. In 1999, Van Dorpe et al. [7] reported that CFT can represent a late stage of inflammatory myofibroblastic tumour (IMT) as the histology showing features of both. Histology of IMT lesions is commonly more cellular, less hyalinised and lack in calcification [8]. CFT has also been reported to be associated with sclerosing Figure 2: MRI image of a splenic mass angiomatoid nodular transformation (SANT) of the spleen [9, 10]. This is a rare benign lesion of the spleen with unknown aetiology. The nodules of SANT are thought to show abnormal reactionary transformation of red pulp triggered by an exaggeration of stro- of the splenic lesion at 6 cm (Figs 1 and 2). The CT scan also mal response, making it more likely to be reactive rather than picked up numerous small calcifications scattered throughout neoplastic [9]. A splenectomy would be required for the definitive the peritoneal cavity. diagnosis of SANT as a core biopsy will be inadequate. She then had a diagnostic laparoscopy with a view of Ideally, local CFT can be resected when diagnosed [5, 8]but performing a splenectomy. However, widespread nodules were in cases of multifocal CFT this might prove a challenge. Some found in the peritoneum and throughout the abdomen as argued that further resection of other masses will not be ben- shown in Fig. 3. There was no evidence of malignancy found eficial if the patients are asymptomatic [8]. Generally, CFT has on laparoscopy. Given these findings, further biopsies of the good prognosis due to its benign nature. However, there has been nodules were obtained and splenectomy was abandoned. documentation of a few cases of patients with recurrence during As the investigations thus far were non-conclusive, positron the follow-up period [2, 5]. There has been no mortality reported emission tomography (PET) scan was performed while awaiting due to CFT [5]. for the result of the peritoneal biopsies. This was performed to look for possible primary malignancy. The splenic lesion demon- strated low-grade activity, below that typically seen in high- CONCLUSION grade haematological malignancy as seen on Fig. 4. Combined with calcifications throughout the peritoneal cavity, an inflam- CFT is a rare benign tumour with non-specific anatomical dis- matory or infective cause would be more likely. tribution. Due to this, patients tend to present with non-specific The peritoneal nodules biopsies were reported as well- symptoms and the tumour is usually picked up on radiological marginated with prominent areas of hyalinization and numer- imaging. Histology is the gold standard to diagnose CFT and this ous foci of psammomatous calcifications. This is consistent with can be confirmed by experienced pathologists. The aetiology of the diagnosis of CFT. With widespread CFT lesions, the patient CFT is still unknown and further studies should be implemented would not benefit from splenectomy. She is being followed up to identify the pathophysiology of the disease. Downloaded from https://academic.oup.com/jscr/article/2021/01/rjaa573/6102033 by DeepDyve user on 26 January 2021 Calcifying fibrous tumour 3 Figure 3: (a and b) Nodules found on small bowel mesentery. (c and d) Nodules on the stomach. (e) Nodule on the peritoneal cavity. 2. Fetsch JF, Montgomery EA, Meis JM. Calcifying fibrous pseu- dotumor. Am J Surg Pathol 1993;17:502–8. 3. Nascimento AF, Ruiz R, Hornick JL, Fletcher CDM. Calcifying fibrous ‘pseudotumor’: Clinicopathologic study of 15 cases and analysis of its relationship to inflammatory myofibrob- lastic tumor. Int J Surg Pathol 2002;10:189–96. 4. Fletcher F, Bridge CDM, Hogendoorn JA, Mertens P. WHO classification of tumours of soft tissue. In: WHO Classification of Tumours of Soft Tissue and Bone, Vol. 46, 4th edn. Boston, MA, US, 2013, 10–2. 5. Chorti A, Papavramidis TS, Michalopoulos A. Calcifying fibrous tumor. Med (United States) 2016;95:e3690. 6. Chen KTKMD. Familial peritoneal multifocal calcifying fibrous tumor. Am J Clin Pathol 2003;119:811–5. 7. Van Dorpe J, Ectors N, Geboes K, D’Hoore A, Sciot R. Is calcifying fibrous pseudotumor a late sclerosing stage of inflammatory myofibroblastic tumor? Am J Surg Pathol 1999;23:329–35. 8. Azam F, Chatterjee M, Kelly S, Pinto M, Aurangabadkar A, Latif M, et al. Multifocal calcifying fibrous tumor at six sites in one patient: a case report. World J Surg Oncol 2014;12: 9. Kuo TT, Chen TC, Lee LY. Sclerosing angiomatoid nodular Figure 4: PET scan showing low-grade activity in the spleen transformation of the spleen (SANT): clinicopathological study of 10 cases with or without abdominal disseminated calcifying fibrous tumors, and the presence of a significant number of IgG4+ plasma cells. Pathol Int 2009;59:844–50. REFERENCES 10. Lee JC, Lien HC, Hsiao CH. Coexisting sclerosing angiomatoid 1. Rosenthal NS, Abdul-Karim FW. Childhood fibrous tumor nodular transformation of the spleen with multiple calcify- with psammoma bodies. Clinicopathologic features in two ing fibrous pseudotumors in a patient. J Formos Med Assoc cases. Arch Pathol Lab Med 1988;112:798–800. 2007;106:234–9.
Journal of Surgical Case Reports – Oxford University Press
Published: Jan 1, 2021
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