Cutaneous Metastases of Ampullary Adenocarcinoma: Case report and literature review
Rosales-Sotomayor A1. Castro-Molina SA1. Méndez-Flores S1. Durand-Muñoz 1. Carrillo-Cordova DM1. Rosas-Camargo V2. DominguezCherit J1
1Department of Dermatology. Instituto Nacional de Ciencias Médicas y Nutrición “Salvador Zubirán, Avenida Vasco de Quiroga 15. Colonia Belisario Domínguez Sección XVI. CP 14080. Mexico City, Mexico.
2Department of Oncology. Instituto Nacional de Ciencias Médicas y Nutrición “Salvador Zubirán, Avenida Vasco de Quiroga 15. Colonia Belisario Domínguez Sección XVI. CP 14080. Mexico City, Mexico.
Received Date: 29/09/2022; Published Date: 13/10/2022.
*Corresponding author: *Judith Dominguez-Cherit, Department of Dermatology. Instituto Nacional de Ciencias Médicas y Nutrición “Salvador Zubirán, Avenida Vasco de Quiroga 15. Colonia Belisario Domínguez Sección XVI. CP 14080. Mexico City, Mexico.
Cutaneous metastases occur in 1% to 10% of patients with metastatic disease. Their recognition is important due to prognostic implications. The most frequently observed cutaneous metastatic cancers are breast, lung, colon, and melanoma with a wide variation in morphology of lesions, and generally they imply a poor prognosis. We present a rare case of a 64-year-old woman with cutaneous metastases on the scalp from ampullary carcinoma and long-term follow-up.
Key words : Cutaneous metastasis, cancer, ampullary carcinoma.
A 64-year-old woman known for stage IIA (pT3 pN0 M0) ampullary adenocarcinoma with pancreatobiliary phenotype, underwent Whipple procedure in September 2014 and received gemcitabine as adjuvant chemotherapy. In July 2018, she developed systemic recurrence with mediastinal disease and superior vena cava syndrome and received first-line palliative chemotherapy with cisplatin/5-fluorouracil (5FU), the best response was stable disease. In October 2020 she presented with a 2-month history of rapidly growing scalp lesions on the occipital and left parietal region. Two erythematous nodules, with meliceric crust and firm consistency on palpation were found, the largest one measured 2 cm. (Figure1a, b) Dermoscopy revealed a central ulcer, white and pink areas some structureless areas, serpentine, comma-shaped, and arborizing vessels (Image1c). A skin biopsy showed nests of moderately differentiated neoplastic cells arranged in a glandular pattern (Image1d,e). Immunohistochemical analysis was positive for CK2, CK19, CDX2, but negative for CK20, compatible with pancreatobiliary carcinoma (Figure1f,g,h). After skin involvement was confirmed, she started second-line chemotherapy with irinotecan.
Figure 1a: Image on the occipital region with erythematosus nodule diameter of 2 cm, well defined borders with meliceric and hematic crust and firm consistency and irregular on palpation.
Figure 1b: Dermatoscopy, central ulcer, white and pink areas some structureless areas, as well as serpentine, comma-shaped, and arborizing vessels.
Figure 1c: Biopsy, nests of moderately differentiated neoplastic cells arranged in a glandular pattern.
Figure 1d: Close up of nests of moderately differentiated neoplastic cells.
Figure 1e: CD20 positive
Figure 1f: CK7 positive
Figure 1g: CDX2 positive
Table 1: Cases reported by the literature review.
Ampullary carcinoma represents less than 1% of gastrointestinal cancers. Curative surgery is possible for those with localized disease, however, most of the patients will eventually develop recurrent disease. Palliative chemotherapy has variable response rates ranging from 10-40% and an overall survival of 8.1-20.4 months1.
Incidence depends upon the particular malignancy. The highest incidence is observed in metastatic melanoma in which up to 45% of patients can develop skin metastases2. They may appear as multiple or single nodules, plaques, ulcers, inflammatory or sclerodermoid lesions, it may be the first sign of recurrent disease, with 75% of patients also having visceral metastases.3 Schulman et al4 recently reported the variation in the local immunologic environment. They demonstrated that regulatory T cell (Treg) density and CD4:CD8 ratio vary across regions. Since Tregs are localized in hair follicles, areas with relatively high Treg density and increased CD4:CD8 ratio include the head and neck. These areas may be permissive to tumor growth, making them sites most frequently affected per surface area unit. Dissemination may occur hematogenously or through the lymphatics, it has been suggested that the vascularity of the scalp and possible dissemination through the vertebral venous system may be contributing factors.
On dermoscopy skin metastases show a high prevalence of a vascular pattern composed mainly by serpentine or linear irregular vessels, followed by arborizing vessels, and polymorphous pattern, consistent with the ability of a tumor to produce and recruit new capillaries.3
There are only a few cases of cutaneous metastases of ampullary carcinoma, most of them involved the head and neck except for the case reported by Cho et al5, in which the thumb and middle finger were affected. Recently, Fernandez-Flores6 described the case of a 57-year-old woman with a scalp metastasis diagnosed 4 years before, she died 2 years after the diagnosis from widespread disease.
Cutaneous metastasis can cause considerable morbidity secondary to infection, bleeding, disfigurement and pain. Systemic therapy alone often has limited efficacy but skin directed therapy may be potentially useful for symptom palliation, although it will not change the prognosis.
In our case, cutaneous metastases were seen 50 months after initial diagnosis. It suggests that skin involvement could see more frequently in long-term survivors. This emphasizes the importance of dermatological examination in these patients in order to identify and treat appropriately.
In conclusion, we presented a case with unusual metastatic spread in a patient with ampullary carcinoma. Despite the rarity, it is relevant to take into account that some patients with prolonged survival may develop skin metastases and is mandatory a high index of suspicion.
- Valle, J. et al. Cisplatin plus Gemcitabine versus Gemcitabine for Biliary Tract Cancer. New England Journal of Medicine 362, 1273–1281 (2010).
- Lookingbill, D. P., Spangler, N. & Helm, K. F. Cutaneous metastases in patients with metastatic carcinoma: A retrospective study of 4020 patients. J Am Acad Dermatol 29, 228–236 (1993).
- Chernoff, K. A. et al. Dermoscopic findings in cutaneous metastases. JAMA Dermatology 150, 429–433 (2014).
- Schulman, J. M. et al. The distribution of cutaneous metastases correlates with local immunologic milieu. Journal of the American Academy of Dermatology 74, 470–476 (2016).
- Eun, Y. C. et al. Acral metastasis in a patient with ampullary carcinoma. Korean Journal of Internal Medicine 22, 55–58 (2007).
- Fernandez-Flores, A. & Cassarino, D. S. Cutaneous metastasis of adenocarcinoma of the ampulla of vater. American Journal of Dermatopathology 40, 758–761 (2018).