Primary cutaneous adenoid cystic carcinoma explained

Specialty:Dermatology

Primary cutaneous adenoid cystic carcinoma is a cutaneous condition characterized by a tumor that usually presents on the chest, scalp, or vulva of middle- to older-aged persons.[1] Primary cutaneous adenoid cystic carcinomas have been misinterpreted as metastatic lesions.[2] It was characterized in 1975.[3]

Primary cutaneous adenoid cystic carcinoma is a hard, slowly expanding, ill-defined tumor causing discomfort, itching, and secondary baldness, or may be asymptomatic.

Primary cutaneous adenoid cystic carcinoma is a rare condition that is believed to be caused by somatic mutations.

Primary cutaneous adenoid cystic carcinoma diagnosis relies on tumor histology features, but a comprehensive clinical and radiographic examination is necessary to identify other primary disease indications, especially in salivary glands.

Primary cutaneous adenoid cystic carcinoma therapy involves broad surgical excision with a 2 cm safety margin, and lymphadenectomy if nodal involvement is suspected.

Signs and symptoms

Primary cutaneous adenoid cystic carcinoma frequently manifests as a hard, slowly expanding, ill-defined nodule or tumor that can cause symptoms including discomfort, itching, and secondary baldness, or it might be asymptomatic.[4] The locations of predilection have been the chest and scalp (at least 40%).[5]

Causes

It's uncertain what causes primary cutaneous adenoid cystic carcinoma. They most likely stem from somatic mutations.[6] Somatic mutation patterns haven't been studied, though.[7] Histopathology indicates that a common developmental mechanism is shared by salivary and primary cutaneous adenoid cystic carcinoma, since many of them exhibited high levels of MYB by immunohistochemistry or carried the fusion gene MYB-NFIB.[8]

Diagnosis

Due to the lack of distinct clinical signs in primary cutaneous adenoid cystic carcinoma, the diagnosis is generally made based on the features of the tumor's histology. A thorough clinical and radiographic examination must be performed in order to search for other indications of primary disease, particularly in the salivary glands.[6]

Treatment

In order to prevent the tumor's frequent recurrence, the usual therapy for primary cutaneous adenoid cystic carcinoma involves a broad surgical excision with at least a 2 cm safety margin from the tumor.[9] When there is a surgical or clinical suspicion of nodal involvement, lymphadenectomy is carried out.[10]

See also

Further reading

External links

Notes and References

  1. Book: James, William D. . Berger, Timothy G.. Andrews' Diseases of the Skin: Clinical Dermatology . Saunders Elsevier . 2006 . 0-7216-2921-0 . etal.
  2. Book: Rapini, Ronald P. . Bolognia, Jean L. . Jorizzo, Joseph L. . Dermatology: 2-Volume Set . Mosby . St. Louis . 2007 . 1710 . 978-1-4160-2999-1 .
  3. Naylor E, Sarkar P, Perlis CS, Giri D, Gnepp DR, Robinson-Bostom L . Primary cutaneous adenoid cystic carcinoma . J. Am. Acad. Dermatol. . 58 . 4 . 636–41 . April 2008 . 18342709 . 10.1016/j.jaad.2007.12.005 .
  4. Tiwari . Raja . Agarwal . Savita . Sharma . Manik . Gaba . Sunil . Primary cutaneous adenoid cystic carcinoma: A clinical and histopathological mimic: A case report . Oral and Maxillofacial Surgery Cases . Elsevier BV . 4 . 4 . 2018 . 2214-5419 . 10.1016/j.omsc.2018.09.002 . 175–179. free .
  5. Ko . Y. H. . Lee . M. A. . Hong . Y. S. . Lee . K. S. . Jung . C.-K. . Kim . Y. S. . Sun . D.-I. . Kim . B. S. . Kim . M. S. . Kang . J. H. . Prognostic Factors Affecting the Clinical Outcome of Adenoid Cystic Carcinoma of the Head and Neck . Japanese Journal of Clinical Oncology . Oxford University Press (OUP) . 37 . 11 . 2007-10-10 . 0368-2811 . 10.1093/jjco/hym119 . 805–811. 18057012 .
  6. Temnithikul . Bhakinai . Rungrunanghiranya . Suthat . Limtanyakul . Piyakan . Jerasuthat . Suthep . Paige . David G. . Primary cutaneous adenoid cystic carcinoma of the scalp: A case report, immunohistochemistry and review of the literature . Skin Health and Disease . Wiley . 2 . 2 . 2022-04-28 . e118 . 2690-442X . 10.1002/ski2.118 . 35677929 . 9168016 .
  7. Rütten . Arno . Hegenbarth . Wolfgang . Kohl . Peter K. . Hillen . Uwe . Redler . Silke . Primary cutaneous adenoid cystic carcinoma mimicking dermal cylindroma: histology of the complete surgical excision as the key to diagnosis . JDDG: Journal der Deutschen Dermatologischen Gesellschaft . Wiley . 16 . 8 . 2018 . 1610-0379 . 10.1111/ddg.13600 . 1016–1018. 30094921 .
  8. North . Jeffrey P. . McCalmont . Timothy H. . Fehr . André . van Zante . Annemieke . Stenman . Göran . LeBoit . Philip E. . Detection of MYB Alterations and Other Immunohistochemical Markers in Primary Cutaneous Adenoid Cystic Carcinoma . American Journal of Surgical Pathology . Ovid Technologies (Wolters Kluwer Health) . 39 . 10 . 2015 . 0147-5185 . 10.1097/pas.0000000000000463 . 1347–1356. 26076064 .
  9. Raychaudhuri . Sujata . Santosh . Kv . Satish Babu . Hv . Primary cutaneous adenoid cystic carcinoma of the chest wall: A rare entity . Journal of Cancer Research and Therapeutics . 8 . 4 . 2012 . 0973-1482 . 10.4103/0973-1482.106583 . free . 633. 23361287 .
  10. Lestouquet . Fabienne Robuschi . Moya . Ana Isabel Sánchez . Guerra . Silvia Honorato . Alzate . Cristina Janeth Cardona . Primary cutaneous adenoid cystic carcinoma: An unusual case . Dermatology Online Journal . California Digital Library (CDL) . 19 . 1 . 2013 . 1087-2108 . 10.5070/d38w41t9kb .

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