Low-Grade Neuroendocrine Carcinoma of the Skin (Primary Cutaneous Carcinoid Tumor) as a Distinctive Entity of Cutaneous Neuroendocrine Tumors: A Clinicopathologic Study of 3 Cases With Literature Review

Goto, Keisuke; Anan, Takashi; Nakatsuka, Takashi; Kaku, Yo; Sakurai, Takaki; Fukumoto, Takaya; Kimura, Tetsunori; Shibata, Arihiro

doi:10.1097/dad.0000000000000657

Cited by 22 publications

(38 citation statements)

References 31 publications

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“…The 14 cases demonstrate the slow‐growing nature and favorable prognosis of the tumor type, despite the metastatic potential. They include one local recurrence case, two cases with regional lymph node metastasis and two cases with distant metastasis . A patient with regional lymph node metastasis was alive at 6 years after the surgical resection of the primary site and metastatic lymph node .…”

Section: Discussionmentioning

confidence: 99%

“…The tumor exhibits a distinct immunoprofile of positivity for ER, PgR, AR, GCDFP-15, mammaglobin and GATA3, which could be useful for differentiating it from metastatic mammary carcinoma and metastatic carcinoid tumor. 3 Chen et al 4 and Celasco et al 5…”

Section: Discussionmentioning

confidence: 99%

“…The other patient with distant metastasis underwent a surgical resection of the primary site and subsequent chemotherapy. 3 The patient was alive 30 months after the first visit and 24 months after the surgical resection. No patient died due to the disease during the follow up.…”

Section: Also Reported Newmentioning

confidence: 95%

“…2 Recently, 11 cases including eight cases retrieved from past published work were summarized, and the designation of low-grade neuroendocrine carcinoma of the skin (LGNECS) was proposed. 3 Here, we present a new case of LGNECS that occurred as an umbilical nodule and showed lymph node and distant metastases.…”

Section: Introductionmentioning

confidence: 96%

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Case of low‐grade neuroendocrine carcinoma of the skin presenting metastases to lymph nodes and peritoneum

Okabayashi¹,

Nakagawa²,

Shimizu³

et al. 2019

The Journal of Dermatology

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A 61‐year‐old Japanese man had a gradually growing, red‐colored nodule in his umbilicus from 3 years ago. He had no symptoms such as hot flush, diarrhea or wheezing. Computed tomography detected a 3.0 cm × 3.0 cm enhanced nodule on the umbilical portion, inguinal and axillary lymph node swelling, and peritoneal nodules. Upper and lower gastrointestinal endoscopy and cystoscopy did not show any other tumor. We resected the umbilical nodule and subjected the inguinal lymph node and peritoneal nodule to biopsy. Histopathological findings indicated that the cutaneous lesion was composed of variously sized nests that included small, monomorphic, round to polygonal cells, mainly in the dermis to the peritoneum. Mitotic figures were scant. The inguinal lymph node and peritoneal nodule were positive for metastasis. Immunohistochemistry was diffusely positive for cytokeratin (CK)‐7, CD56, chromogranin A, synaptophysin, estrogen receptor‐α, progesterone receptor, GATA3 and carcinoembryonic antigen, and focally positive for mammaglobin and gross cystic disease fluid protein 15. The Ki‐67 labeling index was 1.5%. The patient was diagnosed with a case of low‐grade neuroendocrine carcinoma of the skin (LGNECS) occurring on the umbilicus. This case exhibited distant peritoneal metastasis, as well as inguinal and axillary lymph node metastases; however, the patient is alive without chemotherapy at 23 months after the first visit. LGNECS is a newly proposed, extremely rare entity that has been reported under various names, including primary cutaneous carcinoid tumor. In the present case, this tumor shows a slow‐growing nature and favorable prognosis, even though it harbors metastatic potential.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Also Reported Newmentioning

confidence: 95%

Section: Introductionmentioning

confidence: 96%

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Case of low‐grade neuroendocrine carcinoma of the skin presenting metastases to lymph nodes and peritoneum

Okabayashi¹,

Nakagawa²,

Shimizu³

et al. 2019

The Journal of Dermatology

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“…Skin and soft tissue are exceptional sites of NEN, the majority are actually secondary spreading of priory known or unknown NEN of other locations (8). Little data have been published related to the topic (8,9). Some cases reports showed invasion of dermis and subcutis, probably more frequent in older adults (8,9).…”

Section: General Presentation Malignancy Featuresmentioning

confidence: 99%

Skin involvement in neuroendocrine neoplasia

Valea¹,

Şandru²,

Albu³

et al. 2020

Ro J Med Pract.

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Neuroendocrine neoplasia (NEN), priory known as neuroendocrine tumours represent the modern approach of traditionally named "carcinoid" tumours. We shortly introduce benign and malign dermatological aspects that are linked to NEN field. This is a multidisciplinary perspective. The paper is a mini-review. 30 articles are included as references. NEN represents 2% of all cancers, being considered orphan disease. Skin and soft tissue are exceptional sites of NEN, the majority are actually secondary spreading of NEN of other locations. Little data have been published related to the topic. Some cases reports showed invasion of dermis and subcutis, probably more frequent in older adults. The entity has been named low-grade neuroendocrine carcinoma of the skin, also named primary cutaneous carcinoid tumour. As seen in NEN diagnosis, the immunohistochemistry is essential like chromogranin A, synaptophysin, neuron specific enolase which are classical neuroendocrine markers and non-neuroendocrine parameters are useful for differential diagnosis like estrogens, androgens and progesterone receptors, GATA3, carcinoembryonic antigen. Merkel cell carcinoma represents the high grade NEN. It has a poor prognosis, with a 30% risk of local metastasis. The risk factors are either viral infection like Merkel cell polyomavirus, either long time exposure to ultraviolet light. The prognostic is similar between the two factors. Surgical removal is the first line therapy and adjuvant treatment is radiotherapy and chemotherapy. Skin NEN represents an exceptional finding of aggressive profile. Their prompt recognition may help the prognosis.

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Merkel Cell Carcinoma

Becker,

Brownell,

Kervarrec

2024

Rook's Textbook of Dermatology

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Merkel cell carcinoma (MCC) is a highly aggressive neuroendocrine skin cancer characterised by early and frequent metastasis resulting in a 5‐year disease‐associated mortality rate of more than 30% for all stages combined. Predisposing factors include immune suppression, chronic sun exposure and advanced age. The carcinogenesis of MCC is associated either with the clonal integration of Merkel cell polyomavirus DNA into the host genome or ultraviolet‐induced DNA mutations; both resulting in similar cellular aberrations. While MCC is still a rare cancer, its incidence is rising. Management of the primary tumour stage includes wide local excision, sentinel lymph node biopsy and adjuvant radiation. The main pillar of management of systemic disease is immunotherapy by immune checkpoint inhibition.

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Low-Grade Neuroendocrine Carcinoma of the Skin (Primary Cutaneous Carcinoid Tumor) as a Distinctive Entity of Cutaneous Neuroendocrine Tumors: A Clinicopathologic Study of 3 Cases With Literature Review

Cited by 22 publications

References 31 publications

Case of low‐grade neuroendocrine carcinoma of the skin presenting metastases to lymph nodes and peritoneum

Case of low‐grade neuroendocrine carcinoma of the skin presenting metastases to lymph nodes and peritoneum

Skin involvement in neuroendocrine neoplasia

Merkel Cell Carcinoma

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