Fibrolamellar Hepatocellular Carcinoma: Report of a Rare Case with Rapid Progression and Unusual Metastatic Sites
Abstract
Fibrolamellar hepatocellular carcinoma (FLHCC) is a rare primary liver tumor that affects young adults, with unknown biology. We present a case of 21‐yr‐old female with a liver tumor revealed by hemangioma feature at first presentation, after few months, presented with metastatic liver cancer. In pathologic investigation fibrolamellar hepatocellular carcinoma was confirmed. In spite of liver mass resection and lung metastasectomy, after short time, tumor recurrence was occurred. In 18F-FDG PET-CT scan ,lung, ovary, colon and peritoneal invasion was reported. Unfortunately, The patient died one year after diagnosis with rapid progression and multiple unusual metastatic site. Here, we also review the literature concerning FLHCC in many aspects.
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2. El‐Serag HB, Davila JA. Is fibrolamellar carcinoma different from hepatocellular carcinoma? A US population‐based study. Hepatology. 2004 Mar;39(3):798-803.
3. Moore SW, Millar AJ, Hadley GP, Ionescu G, Kruger M, Poole J, Stones D, Wainwright L, Chitnis M, Wessels G. Hepatocellular carcinoma and liver tumors in South African children: a case for increased prevalence. Cancer: Interdisciplinary International Journal of the American Cancer Society. 2004 Aug 1;101(3):642-9.
4. Torbenson M. Fibrolamellar carcinoma: 2012 update. Scientifica. 2012 Sep 23;2012.
5. Ramai D, Ofosu A, Lai JK, Gao ZH, Adler DG. Fibrolamellar hepatocellular carcinoma: a population-based observational study. Digestive diseases and sciences. 2020 Feb 12:1-7.
6. Honeyman JN, Simon EP, Robine N, Chiaroni-Clarke R, Darcy DG, Lim II, Gleason CE, Murphy JM, Rosenberg BR, Teegan L, Takacs CN. Detection of a recurrent DNAJB1-PRKACA chimeric transcript in fibrolamellar hepatocellular carcinoma. Science. 2014 Feb 28;343(6174):1010-4.
7. Lafaro KJ, Pawlik TM. Fibrolamellar hepatocellular carcinoma: current clinical perspectives. Journal of hepatocellular carcinoma. 2015;2:151.
8. Berman MM, Libbey NP, Foster JH. Hepatocellular carcinoma. Polygonal cell type with fibrous stroma—an atypical variant with a favorable prognosis. Cancer. 1980 Sep 15;46(6):1448-55.
9. Craig JR, Peters RL, Edmondson HA, Omata M. Fibrolamellar carcinoma of the liver: A tumor of adolescents and young adults with distinctive clinico‐pathologic features. Cancer. 1980 Jul 15;46(2):372-9.
10. EDMONDSON HA. Differential diagnosis of tumors and tumor-like lesions of liver in infancy and childhood. AMA journal of diseases of children. 1956 Feb 1;91(2):168-86.
11. Chaudhari VA, Khobragade K, Bhandare M, Shrikhande SV. Management of fibrolamellar hepatocellular carcinoma. Chinese clinical oncology. 2018 Oct 1;7(5):51-.
12. Maniaci V, Davidson BR, Rolles K, Dhillon AP, Hackshaw A, Begent RH, Meyer T. Fibrolamellar hepatocellular carcinoma–Prolonged survival with multimodality therapy. European Journal of Surgical Oncology (EJSO). 2009 Jun 1;35(6):617-21.
13. Ang CS, Kelley RK, Choti MA, Cosgrove DP, Chou JF, Klimstra D, Torbenson MS, Ferrell L, Pawlik TM, Fong Y, O'Reilly EM. Clinicopathologic characteristics and survival outcomes of patients with fibrolamellar carcinoma: data from the fibrolamellar carcinoma consortium. Gastrointestinal cancer research: GCR. 2013 Jan;6(1):3.
14. Ichikawa T, Federle MP, Grazioli L, Marsh W. Fibrolamellar hepatocellular carcinoma: pre-and posttherapy evaluation with CT and MR imaging. Radiology. 2000 Oct;217(1):145-51.
15. Stipa F, Yoon SS, Liau KH, Fong Y, Jarnagin WR, D'Angelica M, Abou‐Alfa G, Blumgart LH, DeMatteo RP. Outcome of patients with fibrolamellar hepatocellular carcinoma. Cancer: Interdisciplinary International Journal of the American Cancer Society. 2006 Mar 15;106(6):1331-8.
16. Kakar S, Burgart LJ, Batts KP, Garcia J, Jain D, Ferrell LD. Clinicopathologic features and survival in fibrolamellar carcinoma: comparison with conventional hepatocellular carcinoma with and without cirrhosis. Modern pathology. 2005 Nov;18(11):1417-23.
17. Hemming AW, Langer B, Sheiner P, Greig PD, Taylor BR. Aggressive surgical management of fibrolamellar hepatocellular carcinoma. Journal of Gastrointestinal Surgery. 1997 Jul 1;1(4):342-6.
18. Benito V, Segura J, Martínez MS, Arencibia O, Lubrano A. Fibrolamellar hepatocellular carcinoma metastatic to the ovary. Journal of Obstetrics and Gynaecology. 2012 Feb 1;32(2):200-2.
19. Chakrabarti S, Tella SH, Kommalapati A, Huffman BM, Yadav S, Riaz IB, Goyal G, Mody K, Borad M, Cleary S, Smoot RL. Clinicopathological features and outcomes of fibrolamellar hepatocellular carcinoma. Journal of gastrointestinal oncology. 2019 Jun;10(3):554.
20. Do RK, McErlean A, Ang CS, DeMatteo RP, Abou-Alfa GK. CT and MRI of primary and metastatic fibrolamellar carcinoma: a case series of 37 patients. The British journal of radiology. 2014 Aug;87(1040):20140024.
21. Merani S, Lees GM. Repeated metastasectomy in an adolescent with fibrolamellar hepatocellular carcinoma: A case report. Journal of Pediatric Surgery Case Reports. 2016 May 1;8:3-6.
22. Thirabanjasak D, Sosothikul D, Mahayosnond A, Thorner PS. Fibrolamellar carcinoma presenting as a pancreatic mass: case report and review of the literature. Journal of pediatric hematology/oncology. 2009 May 1;31(5):370-2.
23. Villa NA, Pannala R, Faigel DO, Haakinson DJ, Katariya N, Ramanathan R, Jaroszewski D, Lidner TK, Byrne T. Metastatic fibrolamellar hepatocellular carcinoma to the pancreas. Case reports in gastroenterology. 2015;9(2):266-71.
24. Wojcicki M, Lubikowski J, Post M, Chmurowicz T, Wiechowska-Kozlowska A, Krawczyk M. Aggressive surgical management of recurrent lymph node and pancreatic head metastases of resected fibrolamellar hepatocellular carcinoma: a case report. JOP. Journal of the Pancreas. 2012 Sep 10;13(5):529-32.
25. Vaideeswar P, Pandit MJ, Deshpande JR, Sivaraman A, Vora IM. Fibrolamellar carcinoma of the liver--an unusual presentation. Journal of postgraduate medicine. 1993 Jul 1;39(3):159-61.
26. Hammond WJ, Lalazar G, Saltsman JA, Farber BA, Danzer E, Sherpa TC, Banda CD, Andolina JR, Karimi S, Brennan CW, Torbenson MS. Intracranial metastasis in fibrolamellar hepatocellular carcinoma. Pediatric blood & cancer. 2018 Apr;65(4):e26919.
27. Bilbao I, Vilallonga R, Allende E, Montero Á, Quiroga S, Viladomiu L, Charco R, Margarit C. Krukenberg's tumor as the first clinical manifestation of fibrolamellar hepatocarcinoma. Gastroenterologia y hepatologia. 2008 Jun 1;31(6):341-6.
28. Montero A, Allende H, Tallada N, y Cajal SR, Margarit C, Viladomiu L. Fine needle aspiration cytology of massive bilateral ovarian metastasis of fibrolamellar hepatocellular carcinoma. Acta cytologica. 2007;51(4):682-3.
29. Ciurea SH, Matei E, Stanescu CS, Lupescu IG, Boros M, Herlea V, Luca NI, Dorobantu BM. Fibrolamellar hepatocellular carcinoma with ovarian metastasis-an unusual presentation. Rom J Morphol Embryol. 2017 Jan 1;58(1):187-92.
30. Marion Lee M, Sanford Silverman M, Hans Hansen M, Vikram Patel M, Laxmaiah Manchikanti MD. A comprehensive review of opioid-induced hyperalgesia. Pain physician. 2011 Mar;14:145-61.
31. Dülger AC, Atmaca M, Aytemiz E, Altındal E, Akdeniz H. A Case of Fibrolamellar Hepatocellular Carcinoma Presented with Elevated Serum Vitamın B12 Levels. Eur J Gen Med. 2011;8(3):250-2.
32. Ermens AA, Vlasveld LT, Lindemans J. Significance of elevated cobalamin (vitamin B12) levels in blood. Clinical biochemistry. 2003 Nov 1;36(8):585-90.
33. Lildballe DL, Nguyen KQ, Poulsen SS, Nielsen HO, Nexo E. Haptocorrin as marker of disease progression in fibrolamellar hepatocellular carcinoma. European Journal of Surgical Oncology (EJSO). 2011 Jan 1;37(1):72-9.
34. Mavros MN, Mayo SC, Hyder O, Pawlik TM. A systematic review: treatment and prognosis of patients with fibrolamellar hepatocellular carcinoma. Journal of the American College of Surgeons. 2012 Dec 1;215(6):820-30.
35. Zakka K, Jiang R, Alese OB, Shaib WL, Wu C, Wedd JP, Sellers MT, Behera M, El-Rayes BF, Akce M. Clinical outcomes of rare hepatocellular carcinoma variants compared to pure hepatocellular carcinoma. Journal of hepatocellular carcinoma. 2019;6:119.
36. Vilgrain V, Boulos L, Vullierme MP, Denys A, Terris B, Menu Y. Imaging of atypical hemangiomas of the liver with pathologic correlation. Radiographics. 2000 Mar;20(2):379-97.
37. Liu S, Chan KW, Wang B, Qiao L. Fibrolamellar hepatocellular carcinoma. American Journal of Gastroenterology. 2009 Oct 1;104(10):2617-24.
38. Minhas P, Singh J, Prasad R, Virmani S. Fibrolamellar HCC: A case based pictorial review highlighting the role of 18F-FDG PET CT with multimodality comparison. Journal of Nuclear Medicine. 2020 May 1;61(supplement 1):1188-.
2. El‐Serag HB, Davila JA. Is fibrolamellar carcinoma different from hepatocellular carcinoma? A US population‐based study. Hepatology. 2004 Mar;39(3):798-803.
3. Moore SW, Millar AJ, Hadley GP, Ionescu G, Kruger M, Poole J, Stones D, Wainwright L, Chitnis M, Wessels G. Hepatocellular carcinoma and liver tumors in South African children: a case for increased prevalence. Cancer: Interdisciplinary International Journal of the American Cancer Society. 2004 Aug 1;101(3):642-9.
4. Torbenson M. Fibrolamellar carcinoma: 2012 update. Scientifica. 2012 Sep 23;2012.
5. Ramai D, Ofosu A, Lai JK, Gao ZH, Adler DG. Fibrolamellar hepatocellular carcinoma: a population-based observational study. Digestive diseases and sciences. 2020 Feb 12:1-7.
6. Honeyman JN, Simon EP, Robine N, Chiaroni-Clarke R, Darcy DG, Lim II, Gleason CE, Murphy JM, Rosenberg BR, Teegan L, Takacs CN. Detection of a recurrent DNAJB1-PRKACA chimeric transcript in fibrolamellar hepatocellular carcinoma. Science. 2014 Feb 28;343(6174):1010-4.
7. Lafaro KJ, Pawlik TM. Fibrolamellar hepatocellular carcinoma: current clinical perspectives. Journal of hepatocellular carcinoma. 2015;2:151.
8. Berman MM, Libbey NP, Foster JH. Hepatocellular carcinoma. Polygonal cell type with fibrous stroma—an atypical variant with a favorable prognosis. Cancer. 1980 Sep 15;46(6):1448-55.
9. Craig JR, Peters RL, Edmondson HA, Omata M. Fibrolamellar carcinoma of the liver: A tumor of adolescents and young adults with distinctive clinico‐pathologic features. Cancer. 1980 Jul 15;46(2):372-9.
10. EDMONDSON HA. Differential diagnosis of tumors and tumor-like lesions of liver in infancy and childhood. AMA journal of diseases of children. 1956 Feb 1;91(2):168-86.
11. Chaudhari VA, Khobragade K, Bhandare M, Shrikhande SV. Management of fibrolamellar hepatocellular carcinoma. Chinese clinical oncology. 2018 Oct 1;7(5):51-.
12. Maniaci V, Davidson BR, Rolles K, Dhillon AP, Hackshaw A, Begent RH, Meyer T. Fibrolamellar hepatocellular carcinoma–Prolonged survival with multimodality therapy. European Journal of Surgical Oncology (EJSO). 2009 Jun 1;35(6):617-21.
13. Ang CS, Kelley RK, Choti MA, Cosgrove DP, Chou JF, Klimstra D, Torbenson MS, Ferrell L, Pawlik TM, Fong Y, O'Reilly EM. Clinicopathologic characteristics and survival outcomes of patients with fibrolamellar carcinoma: data from the fibrolamellar carcinoma consortium. Gastrointestinal cancer research: GCR. 2013 Jan;6(1):3.
14. Ichikawa T, Federle MP, Grazioli L, Marsh W. Fibrolamellar hepatocellular carcinoma: pre-and posttherapy evaluation with CT and MR imaging. Radiology. 2000 Oct;217(1):145-51.
15. Stipa F, Yoon SS, Liau KH, Fong Y, Jarnagin WR, D'Angelica M, Abou‐Alfa G, Blumgart LH, DeMatteo RP. Outcome of patients with fibrolamellar hepatocellular carcinoma. Cancer: Interdisciplinary International Journal of the American Cancer Society. 2006 Mar 15;106(6):1331-8.
16. Kakar S, Burgart LJ, Batts KP, Garcia J, Jain D, Ferrell LD. Clinicopathologic features and survival in fibrolamellar carcinoma: comparison with conventional hepatocellular carcinoma with and without cirrhosis. Modern pathology. 2005 Nov;18(11):1417-23.
17. Hemming AW, Langer B, Sheiner P, Greig PD, Taylor BR. Aggressive surgical management of fibrolamellar hepatocellular carcinoma. Journal of Gastrointestinal Surgery. 1997 Jul 1;1(4):342-6.
18. Benito V, Segura J, Martínez MS, Arencibia O, Lubrano A. Fibrolamellar hepatocellular carcinoma metastatic to the ovary. Journal of Obstetrics and Gynaecology. 2012 Feb 1;32(2):200-2.
19. Chakrabarti S, Tella SH, Kommalapati A, Huffman BM, Yadav S, Riaz IB, Goyal G, Mody K, Borad M, Cleary S, Smoot RL. Clinicopathological features and outcomes of fibrolamellar hepatocellular carcinoma. Journal of gastrointestinal oncology. 2019 Jun;10(3):554.
20. Do RK, McErlean A, Ang CS, DeMatteo RP, Abou-Alfa GK. CT and MRI of primary and metastatic fibrolamellar carcinoma: a case series of 37 patients. The British journal of radiology. 2014 Aug;87(1040):20140024.
21. Merani S, Lees GM. Repeated metastasectomy in an adolescent with fibrolamellar hepatocellular carcinoma: A case report. Journal of Pediatric Surgery Case Reports. 2016 May 1;8:3-6.
22. Thirabanjasak D, Sosothikul D, Mahayosnond A, Thorner PS. Fibrolamellar carcinoma presenting as a pancreatic mass: case report and review of the literature. Journal of pediatric hematology/oncology. 2009 May 1;31(5):370-2.
23. Villa NA, Pannala R, Faigel DO, Haakinson DJ, Katariya N, Ramanathan R, Jaroszewski D, Lidner TK, Byrne T. Metastatic fibrolamellar hepatocellular carcinoma to the pancreas. Case reports in gastroenterology. 2015;9(2):266-71.
24. Wojcicki M, Lubikowski J, Post M, Chmurowicz T, Wiechowska-Kozlowska A, Krawczyk M. Aggressive surgical management of recurrent lymph node and pancreatic head metastases of resected fibrolamellar hepatocellular carcinoma: a case report. JOP. Journal of the Pancreas. 2012 Sep 10;13(5):529-32.
25. Vaideeswar P, Pandit MJ, Deshpande JR, Sivaraman A, Vora IM. Fibrolamellar carcinoma of the liver--an unusual presentation. Journal of postgraduate medicine. 1993 Jul 1;39(3):159-61.
26. Hammond WJ, Lalazar G, Saltsman JA, Farber BA, Danzer E, Sherpa TC, Banda CD, Andolina JR, Karimi S, Brennan CW, Torbenson MS. Intracranial metastasis in fibrolamellar hepatocellular carcinoma. Pediatric blood & cancer. 2018 Apr;65(4):e26919.
27. Bilbao I, Vilallonga R, Allende E, Montero Á, Quiroga S, Viladomiu L, Charco R, Margarit C. Krukenberg's tumor as the first clinical manifestation of fibrolamellar hepatocarcinoma. Gastroenterologia y hepatologia. 2008 Jun 1;31(6):341-6.
28. Montero A, Allende H, Tallada N, y Cajal SR, Margarit C, Viladomiu L. Fine needle aspiration cytology of massive bilateral ovarian metastasis of fibrolamellar hepatocellular carcinoma. Acta cytologica. 2007;51(4):682-3.
29. Ciurea SH, Matei E, Stanescu CS, Lupescu IG, Boros M, Herlea V, Luca NI, Dorobantu BM. Fibrolamellar hepatocellular carcinoma with ovarian metastasis-an unusual presentation. Rom J Morphol Embryol. 2017 Jan 1;58(1):187-92.
30. Marion Lee M, Sanford Silverman M, Hans Hansen M, Vikram Patel M, Laxmaiah Manchikanti MD. A comprehensive review of opioid-induced hyperalgesia. Pain physician. 2011 Mar;14:145-61.
31. Dülger AC, Atmaca M, Aytemiz E, Altındal E, Akdeniz H. A Case of Fibrolamellar Hepatocellular Carcinoma Presented with Elevated Serum Vitamın B12 Levels. Eur J Gen Med. 2011;8(3):250-2.
32. Ermens AA, Vlasveld LT, Lindemans J. Significance of elevated cobalamin (vitamin B12) levels in blood. Clinical biochemistry. 2003 Nov 1;36(8):585-90.
33. Lildballe DL, Nguyen KQ, Poulsen SS, Nielsen HO, Nexo E. Haptocorrin as marker of disease progression in fibrolamellar hepatocellular carcinoma. European Journal of Surgical Oncology (EJSO). 2011 Jan 1;37(1):72-9.
34. Mavros MN, Mayo SC, Hyder O, Pawlik TM. A systematic review: treatment and prognosis of patients with fibrolamellar hepatocellular carcinoma. Journal of the American College of Surgeons. 2012 Dec 1;215(6):820-30.
35. Zakka K, Jiang R, Alese OB, Shaib WL, Wu C, Wedd JP, Sellers MT, Behera M, El-Rayes BF, Akce M. Clinical outcomes of rare hepatocellular carcinoma variants compared to pure hepatocellular carcinoma. Journal of hepatocellular carcinoma. 2019;6:119.
36. Vilgrain V, Boulos L, Vullierme MP, Denys A, Terris B, Menu Y. Imaging of atypical hemangiomas of the liver with pathologic correlation. Radiographics. 2000 Mar;20(2):379-97.
37. Liu S, Chan KW, Wang B, Qiao L. Fibrolamellar hepatocellular carcinoma. American Journal of Gastroenterology. 2009 Oct 1;104(10):2617-24.
38. Minhas P, Singh J, Prasad R, Virmani S. Fibrolamellar HCC: A case based pictorial review highlighting the role of 18F-FDG PET CT with multimodality comparison. Journal of Nuclear Medicine. 2020 May 1;61(supplement 1):1188-.
| Files | ||
| Issue | Vol 12 No 4 (2020) | |
| Section | Case Reports | |
| DOI | https://doi.org/10.18502/bccr.v12i4.7980 | |
| Keywords | ||
| Fibrolamellar Hepatocellular Carcinoma HCC Metastasis | ||
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How to Cite
1.
Shirvan A, Sotoudeh S, Tahmasebi M. Fibrolamellar Hepatocellular Carcinoma: Report of a Rare Case with Rapid Progression and Unusual Metastatic Sites. Basic Clin Cancer Res. 2021;12(4):201-206.
