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04 April 2021: Articles

Giant Adrenocortical Carcinoma: A Case Report and Review of the Relevant Literature

Unusual or unexpected effect of treatment, Rare disease, Educational Purpose (only if useful for a systematic review or synthesis)

Ioannis Mantzoros A , Stefanos Bitsianis A , Lydia Loutzidou B , Georgios Ntampakis B , Christos Chatzakis C , Panagiotis Christidis B* , Anna Gkiouliava A , Eleni Koraki A , Stamatios Aggelopoulos E

DOI: 10.12659/AJCR.928875

Am J Case Rep 2021; 22:e928875

Table 1. Giant ACCs reported in the literature [3–5,7,10–19].

AuthorAgeSexPresentationLaboratory studiesTumor size (cm)SiteHistological findingsImmunohistochemical studies
Alastrue Vidal et al45FVirilizationElevated: T, DHEA-S16.2RACCN/R
Alastrue Vidal et al50FVirilizationElevated: T, DHEA-S20.3RACCN/R
Almarzouq et al30FAbdominal pain, weight lossNormal20LACCVimentin(+),Synaptophysin(+)
Bacalbasa et al65MCaval compression syndrome, abdominal painN/R35RACCVimentin(+), Synaptophysin(+), Melan-A(+), Calretinin(+), Ki-67(+) (14%)
Bagchi et al35FAltered menstrual symptomsElevated: serum cortisol, norepinephrine, dopamine21LACCN/R
Benassai et al53MPalpable mass on the L flankNormal24LACCN/R
Brown and Bacal64MAbdominal distentionNormal19RACCN/R
Chentli et al34FCushingElevated: Serum GLC, E2, T, 17OH P, CA125, Decreased: ACTH, K+, Hb14.5R ovaryACCInhibin-A(+),Melan-A(+), SF1(+)
Chung et al36FIncidental findingElevated: DHEA-S, Urinary Free Cortisol, 17-KS Decreased: ACTH12LACCSynaptophysin(+),CD56(+), Inhibin-A(+),Melan-A(+)
Coli et al75FAbdominal painN/R15LSarcomatoid ACCMNF-116(+), Vimentin(+), Desmin(+), Actin(+), H-Caldesmon(+), Myogenin(+), HMB-45(+)
Fancellu et al41MFeminizationElevated: E2, Cortisol, ACTH Decreased: T, Gonadotropins27LACCMelan-A(+), Synaptophysin(+)
Fernandez et al64FAbdominal painN/R12RACCN/R
Fimmano et al61MN/RN/R24RACCN/R
Fulawka et al27MNon-specificN/R22LACCVimentin(+), Inhibin(+),Synaptophysin(+),BCL-2(+), Calretinin(+)
Ghorayeb et al50MPalpable mass on the L flankElevated: DHEA18LACCKi-67(+) (12%), IGF-2(+), β-Catenin(+)
Habibi et al38FAbdominal pain, palpable massNormal22LACCKi-67(+) (15–20%)
Hatano et al60MFeminizationElevated: E2, Preg, P, DOC, 17OH-P, DHEA-S Decreased: T, LH, FSH13RACCKi-67(+) (18%), SF1(+)
Hoang et al39MAscites, abdominal massN/R14RACCCytokeratin(+), Vimentin(+), Synaptophysin(+)
Hoang et al53FAbdominal painN/R17LACCCytokeratin(+), Vimentin(+), Synaptophysin(+)
Hoang et al58M1 year History of rapidly enlarging adrenal massN/R13RACCCytokeratin(+), Vimentin(+), Synaptophysin(+)
Hsieh et al82FPrimary hyperaldo-steronismElevated: ALDO, Decreased: Plasma renin activity13LMyxoid ACCSynaptophysin(+), Melan-A(+), Vimentin(+)
Kalra et al34MIncidental findingNormal16LACCInhibin-A(+), Melan-A(+)
Kashiwagi et al47FLower back painDecreased: Hb13.5LACCN/R
Khan et al40MIncidental findingNormal30RACCN/R
Kovecsi et al71MWeight loss, epigastric painNormal13RACCVimentin(+), Inhibin(+), Synaptophysin(+), NSE(+), Ki-67(+) (30%)
Kunieda et al52MWeight loss, palpable massElevated: Cortisol, S, DHEA-S, DHEA, 17-KS Decreased: ACTH29RACCN/R
Lee et al61MRight flank painVMA12RSarcomatoid ACCCytokeratin(+), Vimentin(+), NSE(+)
Lee et al21MR flank pain, palpable massN/R21RACCN/R
Meshikhes et al20MR flank pain, palpable massNormal24LACCVimentin(+), Inhibin(+), Cytokeratin(+)
Ohwada et al47FIncidental findingNormal18RACCN/R
Ohwada et al68MIncidental findingNormal16RACCN/R
Ohwada et al62MWeight loss, bilateral Lower extremities edemaNormal20RACCN/R
Ohwada et al43FCushingElevated: 17-OHCS, 17- KS, DHEA-S15RACCN/R
Onkar and Shilpi47MNon-specificNormal22LACCN/R
Permana et al21FVirilizationElevated: T, DHEA-S, E2, Morning Cortisol Decreased: LH, FSH,15.6RACCNSE(+), HEP1(+), CD56(+)
Reyes et al42FRight flank painNormal12RACCN/R
Saeger et al53FIncidental findingN/R13RSarcomatoid ACCβ-Catenin(+), Vimentin(+), Synaptophysin(+), Desmin(+), SF1(+), Melan-A(+), Ki-67(+) (60%)
Sasaki et al45MEpigastric pain, weight lossNormal17LSarcomatoid ACCSynaptophysin(+), Melan-A(+), Vimentin(+), Calretinin(+), Desmin(+), Myogenin(+), Myoglobin(+)
Souto et al54FCushingElevated: DHEA-S, AE, 17OH-P, T, Urinary free cortisol decreased: LH21LACCKi-67(+) (20%)
Straka et al40MPENSE26RACCKi-67(+) (12%)
Sung et al48FPalpable massN/R19RMyxoid ACCKi-67(+) (4%)
Sung et al59FIncidental findingN/R12.5LMyxoid ACCKi-67(+) (5%)
Sung et al48FNon-specificN/R16RMyxoid ACCKi-67(+) (18%)
Sung et al51MNon-specificN/R15RSarcomatoid ACCKi-67(+) (12%)
Tseng et al56MAKI, PENormal24RACCMelan-A(+)
Uruc et al48FAbdominal painElevated: T, DHEA-S23LACCVimentin(+), Synaptophysin(+), Cytokeratin(+), Ki-67(+) (13%)
Veron Esquivel et al39FHTN, HypoK, metabolic alkalosisElevated: ALDO, renin, cortisol, T, AE13RACCN/R
Wei et al53FPalpable massElevated: T, P12LACCN/R
Wilkinson et al64FAbdominal painNormal12LACCN/R
Wolf et al46MFeminization, varicocele LElevated: P2, E, 17-OHCS, 17-KS17LACCN/R
Yavascaoglu et al51ML flank pain, weight loss, bilateral leg edemaNormal18LACCN/R
Yeh et al53FVirilizationElevated: T, DHEA-S, AE12RACCN/R
ACC – adrenocortical carcinoma; VMA – vanilmandelic acid; NSE – neuron specific enolase; N/R – not reported; ALDO – aldosterone; AE – androstenedione; T – testosterone; E – estrogens; P2 – pregnadiol; 17-OHCS – 17-hydroxycorticosteroids; 17-KS – 17-Ketosteroids; AKI – acute kidney injury; PE – pulmonary edema; SF1 – steroidogenic factor 1; DHEA-S – dehydroepiandrostenedione-sulfate; IGF-2 – insulin-like growth factor 2; GLC – glucose; P – progesterone; ACTH – adrenocorticotropic hormone; Hb – hemoglobin; Preg – pregnenolone; DOC – deoxycorticosterone; 17OH-P – 17-hydroxyprogesterone; LH – luteinizing hormone; FSH – follicle stimulating hormone, S – 11-Deoxycortisol.

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American Journal of Case Reports eISSN: 1941-5923
American Journal of Case Reports eISSN: 1941-5923