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Squamous cell carcinoma of the skin

MedGen UID:
107512
Concept ID:
C0553723
Neoplastic Process
Synonym: Squamous cell skin carcinoma
SNOMED CT: Squamous cell carcinoma of skin (254651007); SCC - Cutaneous squamous cell carcinoma (254651007); Cutaneous squamous cell carcinoma (254651007); SCC - Squamous cell carcinoma of skin (254651007); Spinous cell carcinoma (254651007)
 
HPO: HP:0006739
Monarch Initiative: MONDO:0002529

Definition

Squamous cell carcinoma of the skin is a malignant tumor of squamous epithelium. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • Squamous cell carcinoma of the skin

Conditions with this feature

Xeroderma pigmentosum group A
MedGen UID:
82775
Concept ID:
C0268135
Disease or Syndrome
Xeroderma pigmentosum (XP) is characterized by: Acute sun sensitivity (severe sunburn with blistering, persistent erythema on minimal sun exposure) with marked freckle-like pigmentation of the face before age two years; Sunlight-induced ocular involvement (photophobia, severe keratitis, atrophy of the skin of the lids, ocular surface neoplasms); Greatly increased risk of sunlight-induced cutaneous neoplasms (basal cell carcinoma, squamous cell carcinoma, melanoma) within the first decade of life. Approximately 25% of affected individuals have neurologic manifestations (acquired microcephaly, diminished or absent deep tendon stretch reflexes, progressive sensorineural hearing loss, progressive cognitive impairment, and ataxia). The most common causes of death are skin cancer, neurologic degeneration, and internal cancer. The median age at death in persons with XP with neurodegeneration (29 years) was found to be younger than that in persons with XP without neurodegeneration (37 years).
Xeroderma pigmentosum group B
MedGen UID:
78643
Concept ID:
C0268136
Disease or Syndrome
Xeroderma pigmentosum (XP) is characterized by: Acute sun sensitivity (severe sunburn with blistering, persistent erythema on minimal sun exposure) with marked freckle-like pigmentation of the face before age two years; Sunlight-induced ocular involvement (photophobia, severe keratitis, atrophy of the skin of the lids, ocular surface neoplasms); Greatly increased risk of sunlight-induced cutaneous neoplasms (basal cell carcinoma, squamous cell carcinoma, melanoma) within the first decade of life. Approximately 25% of affected individuals have neurologic manifestations (acquired microcephaly, diminished or absent deep tendon stretch reflexes, progressive sensorineural hearing loss, progressive cognitive impairment, and ataxia). The most common causes of death are skin cancer, neurologic degeneration, and internal cancer. The median age at death in persons with XP with neurodegeneration (29 years) was found to be younger than that in persons with XP without neurodegeneration (37 years).
Keratoderma with scleroatrophy of the extremities
MedGen UID:
98360
Concept ID:
C0406767
Congenital Abnormality
Huriez syndrome (HRZ) is characterized by the triad of congenital scleroatrophy of the distal extremities, palmoplantar keratoderma, and hypoplastic nail changes. The development of aggressive squamous cell carcinoma (SCC) in areas of affected skin is a distinctive feature of the syndrome, occurring in approximately 15% of patients. HRZ-associated SCC shows early onset, mostly in the third to fourth decades of life, and early metastasis formation (summary by Lee et al., 2000). See also 610644 for description of a disorder resembling Huriez syndrome, involving palmoplantar hyperkeratosis and squamous cell carcinoma in association with SRY (480000)-negative female-to-male XX sex reversal, caused by mutation in the RSPO1 gene (609595).
Xeroderma pigmentosum, group E
MedGen UID:
341219
Concept ID:
C1848411
Congenital Abnormality
Xeroderma pigmentosum (XP) is characterized by: Acute sun sensitivity (severe sunburn with blistering, persistent erythema on minimal sun exposure) with marked freckle-like pigmentation of the face before age two years; Sunlight-induced ocular involvement (photophobia, severe keratitis, atrophy of the skin of the lids, ocular surface neoplasms); Greatly increased risk of sunlight-induced cutaneous neoplasms (basal cell carcinoma, squamous cell carcinoma, melanoma) within the first decade of life. Approximately 25% of affected individuals have neurologic manifestations (acquired microcephaly, diminished or absent deep tendon stretch reflexes, progressive sensorineural hearing loss, progressive cognitive impairment, and ataxia). The most common causes of death are skin cancer, neurologic degeneration, and internal cancer. The median age at death in persons with XP with neurodegeneration (29 years) was found to be younger than that in persons with XP without neurodegeneration (37 years).
Xeroderma pigmentosum, group C
MedGen UID:
416702
Concept ID:
C2752147
Disease or Syndrome
Xeroderma pigmentosum (XP) is characterized by: Acute sun sensitivity (severe sunburn with blistering, persistent erythema on minimal sun exposure) with marked freckle-like pigmentation of the face before age two years; Sunlight-induced ocular involvement (photophobia, severe keratitis, atrophy of the skin of the lids, ocular surface neoplasms); Greatly increased risk of sunlight-induced cutaneous neoplasms (basal cell carcinoma, squamous cell carcinoma, melanoma) within the first decade of life. Approximately 25% of affected individuals have neurologic manifestations (acquired microcephaly, diminished or absent deep tendon stretch reflexes, progressive sensorineural hearing loss, progressive cognitive impairment, and ataxia). The most common causes of death are skin cancer, neurologic degeneration, and internal cancer. The median age at death in persons with XP with neurodegeneration (29 years) was found to be younger than that in persons with XP without neurodegeneration (37 years).
Disabling pansclerotic morphea of childhood
MedGen UID:
858243
Concept ID:
C3898649
Disease or Syndrome
Disabling pansclerotic morphea of childhood is the most severe subtype of deep morphea within the spectrum of juvenile localized scleroderma. Patients affected by this systemic inflammatory disorder experience poor wound healing with rapidly progressive deep fibrosis involving the mucous membranes, dermis, subcutaneous fat, fascia, muscles, and bone, leading to contractures, musculoskeletal atrophy, and articular ankylosis. Systemic manifestations include cytopenias and hypogammaglobulinemia, but scleroderma-associated autoantibodies are usually not present. The disorder is associated with high morbidity and mortality due to squamous cell carcinoma, restrictive pulmonary disease, sepsis, and gangrene (Baghdassarian et al., 2023).
Dyskeratosis congenita, autosomal dominant 1
MedGen UID:
1645250
Concept ID:
C4551974
Disease or Syndrome
Dyskeratosis congenita and related telomere biology disorders (DC/TBD) are caused by impaired telomere maintenance resulting in short or very short telomeres. The phenotypic spectrum of telomere biology disorders is broad and includes individuals with classic dyskeratosis congenita (DC) as well as those with very short telomeres and an isolated physical finding. Classic DC is characterized by a triad of dysplastic nails, lacy reticular pigmentation of the upper chest and/or neck, and oral leukoplakia, although this may not be present in all individuals. People with DC/TBD are at increased risk for progressive bone marrow failure (BMF), myelodysplastic syndrome or acute myelogenous leukemia, solid tumors (usually squamous cell carcinoma of the head/neck or anogenital cancer), and pulmonary fibrosis. Other findings can include eye abnormalities (epiphora, blepharitis, sparse eyelashes, ectropion, entropion, trichiasis), taurodontism, liver disease, gastrointestinal telangiectasias, and avascular necrosis of the hips or shoulders. Although most persons with DC/TBD have normal psychomotor development and normal neurologic function, significant developmental delay is present in both forms; additional findings include cerebellar hypoplasia (Hoyeraal Hreidarsson syndrome) and bilateral exudative retinopathy and intracranial calcifications (Revesz syndrome and Coats plus syndrome). Onset and progression of manifestations of DC/TBD vary: at the mild end of the spectrum are those who have only minimal physical findings with normal bone marrow function, and at the severe end are those who have the diagnostic triad and early-onset BMF.
Epidermodysplasia verruciformis, susceptibility to, 2
MedGen UID:
1648344
Concept ID:
C4722258
Finding
Epidermodysplasia verruciformis (EV) is a rare genodermatosis associated with a high risk of skin cancer. EV results from an abnormal susceptibility to specific related human papillomavirus (HPV) genotypes and to the oncogenic potential of some of them, mainly HPV5. Infection with EV-associated HPV leads to the early development of disseminated flat wart-like and pityriasis versicolor-like lesions. Patients are unable to reject their lesions, and cutaneous Bowen carcinomas in situ and invasive squamous cell carcinomas develop in about half of them, mainly on sun-exposed areas (summary by Ramoz et al., 2000). For a discussion of genetic heterogeneity of susceptibility to epidermodysplasia verruciformis, see EV1 (226400).
Epidermodysplasia verruciformis, susceptibility to, 5
MedGen UID:
1648489
Concept ID:
C4749043
Finding
Epidermodysplasia verruciformis-5 is an autosomal recessive immunologic disorder characterized by onset of warts and verrucous or plaque-like skin lesions associated with HPV infection. Immunologic workup shows T-cell lymphopenia, particularly affecting CD4+ T cells. There is an increased risk of skin malignancy, and some patients may have other symptoms of immune dysfunction (summary by Horev et al., 2015). For a discussion of genetic heterogeneity of susceptibility to epidermodysplasia verruciformis, see EV1 (226400).
Dyskeratosis congenita, digenic
MedGen UID:
1823990
Concept ID:
C5774217
Disease or Syndrome
Digenic dyskeratosis congenita (DKCD) is characterized clinically by a combination of mucocutaneous features including abnormal skin pigmentation, nail dystrophy, thin hair, and oral leukoplakia. Some patients may have evidence of bone marrow failure, manifest as immune defects such as recurrent infections or hypogammaglobulinemia. Telomeres are shortened in patient cells. Individuals with DKCD may show severe adverse reactions to treatment with 5-FU (Tummala et al., 2022). For a discussion of genetic heterogeneity of dyskeratosis congenita, see DKCA1 (127550).
Pulmonary fibrosis and/or bone marrow failure syndrome, telomere-related, 7
MedGen UID:
1841121
Concept ID:
C5830485
Disease or Syndrome
Telomere-related pulmonary fibrosis and/or bone marrow failure syndrome-7 (PFBMFT7) is an autosomal dominant disorder characterized by variable manifestations associated with shortened telomeres. Features can include pulmonary fibrosis, emphysema, anemia, lymphopenia, liver involvement with portal hypertension and hepatopulmonary syndrome, premature graying of the hair, nail dystrophy, and predisposition to squamous cell cancers or myelodysplasia (Stanley et al., 2016). For a discussion of genetic heterogeneity of telomere-related pulmonary fibrosis and/or bone marrow failure syndromes, see PFBMFT1 (614742).

Professional guidelines

PubMed

Stratigos AJ, Garbe C, Dessinioti C, Lebbe C, Bataille V, Bastholt L, Dreno B, Concetta Fargnoli M, Forsea AM, Frenard C, Harwood CA, Hauschild A, Hoeller C, Kandolf-Sekulovic L, Kaufmann R, Kelleners-Smeets NWJ, Malvehy J, Del Marmol V, Middleton MR, Moreno-Ramirez D, Pellecani G, Peris K, Saiag P, van den Beuken-van Everdingen MHJ, Vieira R, Zalaudek I, Eggermont AMM, Grob JJ; European Dermatology Forum (EDF), the European Association of Dermato-Oncology (EADO) and the European Organization for Research and Treatment of Cancer (EORTC)
Eur J Cancer 2020 Mar;128:83-102. Epub 2020 Feb 26 doi: 10.1016/j.ejca.2020.01.008. PMID: 32113942
Stratigos AJ, Garbe C, Dessinioti C, Lebbe C, Bataille V, Bastholt L, Dreno B, Fargnoli MC, Forsea AM, Frenard C, Harwood CΑ, Hauschild A, Hoeller C, Kandolf-Sekulovic L, Kaufmann R, Kelleners-Smeets NW, Malvehy J, Del Marmol V, Middleton MR, Moreno-Ramirez D, Pellecani G, Peris K, Saiag P, van den Beuken-van Everdingen MHJ, Vieira R, Zalaudek I, Eggermont AMM, Grob JJ; European Dermatology Forum (EDF), the European Association of Dermato-Oncology (EADO) and the European Organization for Research and Treatment of Cancer (EORTC)
Eur J Cancer 2020 Mar;128:60-82. Epub 2020 Feb 26 doi: 10.1016/j.ejca.2020.01.007. PMID: 32113941
Kallini JR, Hamed N, Khachemoune A
Int J Dermatol 2015 Feb;54(2):130-40. Epub 2014 Nov 27 doi: 10.1111/ijd.12553. PMID: 25428226

Curated

NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®), Squamous Cell Skin Cancer, 2023

Suggested Reading

Recent clinical studies

Etiology

Heppt MV, Leiter U
J Dtsch Dermatol Ges 2023 Apr;21(4):421-424. Epub 2023 Mar 31 doi: 10.1111/ddg.15052. PMID: 36999577
Caudill J, Thomas JE, Burkhart CG
Int J Dermatol 2023 Apr;62(4):483-486. Epub 2022 Mar 24 doi: 10.1111/ijd.16164. PMID: 35324009
Stratigos AJ, Garbe C, Dessinioti C, Lebbe C, Bataille V, Bastholt L, Dreno B, Concetta Fargnoli M, Forsea AM, Frenard C, Harwood CA, Hauschild A, Hoeller C, Kandolf-Sekulovic L, Kaufmann R, Kelleners-Smeets NWJ, Malvehy J, Del Marmol V, Middleton MR, Moreno-Ramirez D, Pellecani G, Peris K, Saiag P, van den Beuken-van Everdingen MHJ, Vieira R, Zalaudek I, Eggermont AMM, Grob JJ; European Dermatology Forum (EDF), the European Association of Dermato-Oncology (EADO) and the European Organization for Research and Treatment of Cancer (EORTC)
Eur J Cancer 2020 Mar;128:83-102. Epub 2020 Feb 26 doi: 10.1016/j.ejca.2020.01.008. PMID: 32113942
Stratigos AJ, Garbe C, Dessinioti C, Lebbe C, Bataille V, Bastholt L, Dreno B, Fargnoli MC, Forsea AM, Frenard C, Harwood CΑ, Hauschild A, Hoeller C, Kandolf-Sekulovic L, Kaufmann R, Kelleners-Smeets NW, Malvehy J, Del Marmol V, Middleton MR, Moreno-Ramirez D, Pellecani G, Peris K, Saiag P, van den Beuken-van Everdingen MHJ, Vieira R, Zalaudek I, Eggermont AMM, Grob JJ; European Dermatology Forum (EDF), the European Association of Dermato-Oncology (EADO) and the European Organization for Research and Treatment of Cancer (EORTC)
Eur J Cancer 2020 Mar;128:60-82. Epub 2020 Feb 26 doi: 10.1016/j.ejca.2020.01.007. PMID: 32113941
Kallini JR, Hamed N, Khachemoune A
Int J Dermatol 2015 Feb;54(2):130-40. Epub 2014 Nov 27 doi: 10.1111/ijd.12553. PMID: 25428226

Diagnosis

Caudill J, Thomas JE, Burkhart CG
Int J Dermatol 2023 Apr;62(4):483-486. Epub 2022 Mar 24 doi: 10.1111/ijd.16164. PMID: 35324009
Stratigos AJ, Garbe C, Dessinioti C, Lebbe C, Bataille V, Bastholt L, Dreno B, Concetta Fargnoli M, Forsea AM, Frenard C, Harwood CA, Hauschild A, Hoeller C, Kandolf-Sekulovic L, Kaufmann R, Kelleners-Smeets NWJ, Malvehy J, Del Marmol V, Middleton MR, Moreno-Ramirez D, Pellecani G, Peris K, Saiag P, van den Beuken-van Everdingen MHJ, Vieira R, Zalaudek I, Eggermont AMM, Grob JJ; European Dermatology Forum (EDF), the European Association of Dermato-Oncology (EADO) and the European Organization for Research and Treatment of Cancer (EORTC)
Eur J Cancer 2020 Mar;128:83-102. Epub 2020 Feb 26 doi: 10.1016/j.ejca.2020.01.008. PMID: 32113942
Stratigos AJ, Garbe C, Dessinioti C, Lebbe C, Bataille V, Bastholt L, Dreno B, Fargnoli MC, Forsea AM, Frenard C, Harwood CΑ, Hauschild A, Hoeller C, Kandolf-Sekulovic L, Kaufmann R, Kelleners-Smeets NW, Malvehy J, Del Marmol V, Middleton MR, Moreno-Ramirez D, Pellecani G, Peris K, Saiag P, van den Beuken-van Everdingen MHJ, Vieira R, Zalaudek I, Eggermont AMM, Grob JJ; European Dermatology Forum (EDF), the European Association of Dermato-Oncology (EADO) and the European Organization for Research and Treatment of Cancer (EORTC)
Eur J Cancer 2020 Mar;128:60-82. Epub 2020 Feb 26 doi: 10.1016/j.ejca.2020.01.007. PMID: 32113941
Stratigos A, Garbe C, Lebbe C, Malvehy J, del Marmol V, Pehamberger H, Peris K, Becker JC, Zalaudek I, Saiag P, Middleton MR, Bastholt L, Testori A, Grob JJ; European Dermatology Forum (EDF); European Association of Dermato-Oncology (EADO); European Organization for Research and Treatment of Cancer (EORTC)
Eur J Cancer 2015 Sep;51(14):1989-2007. Epub 2015 Jul 25 doi: 10.1016/j.ejca.2015.06.110. PMID: 26219687
Kallini JR, Hamed N, Khachemoune A
Int J Dermatol 2015 Feb;54(2):130-40. Epub 2014 Nov 27 doi: 10.1111/ijd.12553. PMID: 25428226

Therapy

Stratigos AJ, Garbe C, Dessinioti C, Lebbe C, Bataille V, Bastholt L, Dreno B, Concetta Fargnoli M, Forsea AM, Frenard C, Harwood CA, Hauschild A, Hoeller C, Kandolf-Sekulovic L, Kaufmann R, Kelleners-Smeets NWJ, Malvehy J, Del Marmol V, Middleton MR, Moreno-Ramirez D, Pellecani G, Peris K, Saiag P, van den Beuken-van Everdingen MHJ, Vieira R, Zalaudek I, Eggermont AMM, Grob JJ; European Dermatology Forum (EDF), the European Association of Dermato-Oncology (EADO) and the European Organization for Research and Treatment of Cancer (EORTC)
Eur J Cancer 2020 Mar;128:83-102. Epub 2020 Feb 26 doi: 10.1016/j.ejca.2020.01.008. PMID: 32113942
Stratigos AJ, Garbe C, Dessinioti C, Lebbe C, Bataille V, Bastholt L, Dreno B, Fargnoli MC, Forsea AM, Frenard C, Harwood CΑ, Hauschild A, Hoeller C, Kandolf-Sekulovic L, Kaufmann R, Kelleners-Smeets NW, Malvehy J, Del Marmol V, Middleton MR, Moreno-Ramirez D, Pellecani G, Peris K, Saiag P, van den Beuken-van Everdingen MHJ, Vieira R, Zalaudek I, Eggermont AMM, Grob JJ; European Dermatology Forum (EDF), the European Association of Dermato-Oncology (EADO) and the European Organization for Research and Treatment of Cancer (EORTC)
Eur J Cancer 2020 Mar;128:60-82. Epub 2020 Feb 26 doi: 10.1016/j.ejca.2020.01.007. PMID: 32113941
Bejar C, Maubec E
Curr Treat Options Oncol 2014 Jun;15(2):302-20. doi: 10.1007/s11864-014-0280-x. PMID: 24643778
DeConti RC
Semin Oncol 2012 Apr;39(2):145-9. doi: 10.1053/j.seminoncol.2012.01.002. PMID: 22484186
Rudolph R, Zelac DE
Plast Reconstr Surg 2004 Nov;114(6):82e-94e. doi: 10.1097/01.prs.0000138243.45735.8a. PMID: 15509920

Prognosis

Heppt MV, Leiter U
J Dtsch Dermatol Ges 2023 Apr;21(4):421-424. Epub 2023 Mar 31 doi: 10.1111/ddg.15052. PMID: 36999577
Caudill J, Thomas JE, Burkhart CG
Int J Dermatol 2023 Apr;62(4):483-486. Epub 2022 Mar 24 doi: 10.1111/ijd.16164. PMID: 35324009
Stratigos AJ, Garbe C, Dessinioti C, Lebbe C, Bataille V, Bastholt L, Dreno B, Fargnoli MC, Forsea AM, Frenard C, Harwood CΑ, Hauschild A, Hoeller C, Kandolf-Sekulovic L, Kaufmann R, Kelleners-Smeets NW, Malvehy J, Del Marmol V, Middleton MR, Moreno-Ramirez D, Pellecani G, Peris K, Saiag P, van den Beuken-van Everdingen MHJ, Vieira R, Zalaudek I, Eggermont AMM, Grob JJ; European Dermatology Forum (EDF), the European Association of Dermato-Oncology (EADO) and the European Organization for Research and Treatment of Cancer (EORTC)
Eur J Cancer 2020 Mar;128:60-82. Epub 2020 Feb 26 doi: 10.1016/j.ejca.2020.01.007. PMID: 32113941
Fu T, Aasi SZ, Hollmig ST
Curr Treat Options Oncol 2016 Jul;17(7):34. doi: 10.1007/s11864-016-0408-2. PMID: 27262708
Kallini JR, Hamed N, Khachemoune A
Int J Dermatol 2015 Feb;54(2):130-40. Epub 2014 Nov 27 doi: 10.1111/ijd.12553. PMID: 25428226

Clinical prediction guides

Stratigos A, Garbe C, Lebbe C, Malvehy J, del Marmol V, Pehamberger H, Peris K, Becker JC, Zalaudek I, Saiag P, Middleton MR, Bastholt L, Testori A, Grob JJ; European Dermatology Forum (EDF); European Association of Dermato-Oncology (EADO); European Organization for Research and Treatment of Cancer (EORTC)
Eur J Cancer 2015 Sep;51(14):1989-2007. Epub 2015 Jul 25 doi: 10.1016/j.ejca.2015.06.110. PMID: 26219687
Bejar C, Maubec E
Curr Treat Options Oncol 2014 Jun;15(2):302-20. doi: 10.1007/s11864-014-0280-x. PMID: 24643778
Rudolph R, Zelac DE
Plast Reconstr Surg 2004 Nov;114(6):82e-94e. doi: 10.1097/01.prs.0000138243.45735.8a. PMID: 15509920
Brash DE, Pontén J
Cancer Surv 1998;32:69-113. PMID: 10489624
Jackson R, Grainge JW
Can Med Assoc J 1975 Sep 6;113(5):396-401. PMID: 125622Free PMC Article

Recent systematic reviews

Piovani D, Hassan C, Repici A, Rimassa L, Carlo-Stella C, Nikolopoulos GK, Riboli E, Bonovas S
Gastroenterology 2022 Sep;163(3):671-684. Epub 2022 May 26 doi: 10.1053/j.gastro.2022.05.038. PMID: 35643170
Green AC, McBride P
BMJ Clin Evid 2014 Aug 18;2014 PMID: 25137222Free PMC Article
Lansbury L, Bath-Hextall F, Perkins W, Stanton W, Leonardi-Bee J
BMJ 2013 Nov 4;347:f6153. doi: 10.1136/bmj.f6153. PMID: 24191270Free PMC Article
Green AC, McBride P
BMJ Clin Evid 2010 May 4;2010 PMID: 21733203Free PMC Article
Lansbury L, Leonardi-Bee J, Perkins W, Goodacre T, Tweed JA, Bath-Hextall FJ
Cochrane Database Syst Rev 2010 Apr 14;2010(4):CD007869. doi: 10.1002/14651858.CD007869.pub2. PMID: 20393962Free PMC Article

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    Curated

    • NCCN, 2023
      NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®), Squamous Cell Skin Cancer, 2023

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