Cutaneous Metastasis of Neuroendocrine Carcinoma with Unknown Primary Site: Case Report and Review of the Literature

Amorim, Gustavo Moreira; Quintella, Danielle Carvalho; Cuzzi, Tullia; Rodrigues, Rosângela Schwarz; Ramos‐e‐Silva, Márcia

doi:10.1159/000440661

Cited by 9 publications

(10 citation statements)

References 35 publications

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“…NETs show a metastatic spread in 30% of cases, more commonly liver, while cutaneous metastases are considered rare [14–16]. In clinical practice, relevant bioclinical features addressing the proper multidisciplinary treatment strategy of neuroendocrine carcinoma consist of morphology, Ki-67 expression, mitoses, functional imaging, and clinical behavior.…”

Section: Introductionmentioning

confidence: 99%

Poorly differentiated neuroendocrine rectal carcinoma with uncommon immune-histochemical features and clinical presentation with a subcutaneous metastasis, treated with first line intensive triplet chemotherapy plus bevacizumab FIr-B/FOx regimen: an experience of multidisciplinary management in clinical practice

et al. 2019

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BackgroundNeuroendocrine tumors (NETs) are heterogeneous, widely distributed tumors arising from neuroendocrine cells. Gastrointestinal (GI)-NETs are the most common and NETs of the rectum represent 15, 2% of gastrointestinal malignancies. Poorly differentiated neuroendocrine carcinomas of the GI tract are uncommon. We report a rare case of poorly differentiated locally advanced rectal neuroendocrine carcinoma with nodal and a subcutaneous metastasis, with a cytoplasmic staining positive for Synaptophysin and Thyroid Transcription Factor-1.Case presentationA 72-year-old male presented to hospital, due to lumbar, abdominal, perineal pain, and severe constipation. A whole-body computed tomography scan showed a mass of the right lateral wall of the rectum, determining significant reduction of lumen caliber. It also showed a subcutaneous metastasis of the posterior abdominal wall. Patient underwent a multidisciplinary evaluation, diagnostic and therapeutic plan was shared and defined. The pathological examination of rectal biopsy and subcutaneous nodule revealed features consistent with small-cell poorly differentiated neuroendocrine carcinoma. First line medical treatment with triplet chemotherapy and bevacizumab, according to FIr-B/FOx intensive regimen, administered for the first time in this young elderly patient affected by metastatic rectal NEC was highly active and tolerable, as previously reported in metastatic colo-rectal carcinoma (MCRC). A consistent rapid improvement in clinical conditions were observed during treatment. After 6 cycles of treatment, CT scan and endoscopic evaluation showed clinical complete response of rectal mass and lymph nodes; patient underwent curative surgery confirming the pathologic complete response at PFS 9 months.Discussion and conclusionsThis case report of a locally advanced rectal NEC with an unusual subcutaneous metastasis deserves further investigation of triplet chemotherapy-based intensive regimens in metastatic GEP NEC.

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Section: Introductionmentioning

confidence: 99%

Poorly differentiated neuroendocrine rectal carcinoma with uncommon immune-histochemical features and clinical presentation with a subcutaneous metastasis, treated with first line intensive triplet chemotherapy plus bevacizumab FIr-B/FOx regimen: an experience of multidisciplinary management in clinical practice

et al. 2019

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“…In line with observations and revisions by Amorim et Al. [16], we remark that the detection of the primary tumor site in metastatic neuroendocrine tumors is a crucial question to improve the choice of therapeutic strategy. Anyhow, for patients with confirmed metastatic disease, systemic therapies and palliative cares of symptoms are the most common approaches [19].…”

Section: Discussionmentioning

confidence: 99%

“…Cases of skin (cutaneous/subcutaneous) metastases derived from neuroendocrine tumors of the lung [3][4][5][6][7][8], gastrointestinal [2,[9][10][11] and genitourinary tract [12][13][14][15] as well as of other origins or with unknown primary tumor site [16][17][18] have been exceptionally reported. In line with observations and revisions by Amorim et Al.…”

Section: Discussionmentioning

confidence: 99%

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Unexpected response to palliative radiotherapy for subcutaneous metastases of an advanced small cell pancreatic neuroendocrine carcinoma: a case report of two different radiation schedules

Ciliberti¹,

Carbonara

Grillo³

et al. 2020

BMC Cancer

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Background: Skin metastases from pancreatic neuroendocrine carcinoma (PNEC) are rare and their palliative treatment is challenging. We report our experience in the multimodal management of one of the few reported cases of metastatic PNEC with multiple visceral and subcutaneous secondary lesions, focusing on the effectiveness of palliative radiotherapy for skin metastases. Case presentation: A 61-years old woman affected by a metastatic PNECwith subcutaneous growing and bleeding secondary lesions (at the scalp, right scapular region and at the back of the left thoracic wall, respectively) obtained a successful control of visceral metastases with the use of chemotherapy and an unexpected local response of her skin metastases with palliative radiotherapy. In particular, two subsequent radiation treatments were performed using different fractionation schedules (30 Gy in 10 fractions and 20 Gy in 5 fractions, respectively). Both radiation treatments were well-tolerated and patient's quality of life was improved. Local response was maintained until patient's deaththat occurred due to cachexia. Conclusions: The presented case highlights the effectiveness and the good tolerance of radiotherapy in the treatment of subcutaneous metastases; nevertheless, further knowledge of the optimal local palliative approach for PNEC metastatic sites is necessary. The experience gained in this work is the occasion to encourage a routine integrated multidisciplinary team management of metastatic PNECs because of their clinical complexity. The aim is to guarantee the optimization of the care with personalized and more effective systemic and local treatmentsalso including supportive cares and treatment-related side effects management.

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“…of the primary site in metastatic NEC can be quite challenging because enlarged lymph nodes tend to be the first and only symptom. The prognosis and survival of patients with NEC is poor [1,2], and so it has been recommended that physical examination as well as biochemical, functional, and imaging tests be performed every 3 months [3]. A thorough physical examination of the skin should also be performed to look for possible metastases.…”

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confidence: 99%