A primary small cell neuroendocrine carcinoma (SCNC) of the oral cavity (cheek mucosa): Description of a case report.

BACKGROUND: Small cell neuroendocrine carcinoma (SCNC) of the oral cavity is a poorly differentiated, high-grade and very aggressive tumor with a poor prognosis. CASE DESCRIPTION: A 64-year-old, Caucasian, smoker man consulted for an ulcero-necrotic, exophytic, lesion of the right retromolar trigone. Haed&neck CT scan showed a right tonsillar tumor lesion. The 18F-PET scan confirmed the presence of a right, highly hypermetabolic tonsillar lesion and two homolateral, cervical lymph nodes. Histology and immunohistochemistry were consisted with the diagnosis of a primary SCNC of the oral cavity. As the tumor was locally advanced and unresectable, the patient underwent a definitive radio-chemotherapy with a cisplatin/etoposide combined regimen (4 cycles). The treatment was well tolerated and led to a complete tumor response. CONCLUSION: The particularity of this case relies on the rarity of the oral SCNC, its difficult and challenging diagnosis, and the complexity of its management that is not validated by large clinical trials, data being extrapolated from small cell lung cancer. In our case, the patient presenting a locally advanced tumor was treated by a combined radio-chemiotherapy leading to a complete tumor regression. The patient's follow up is too short to assess the real benefit of this treatment on overall survival.

A fatal primary gastric melanoma treated by a double immunotherapy with ipilimumab/nivolumab: description of a case report.

Background: Primary gastric melanoma (GM) is a very uncommon tumor with a poor prognosis. Until now, only a few cases have been reported in the literature. Case Description: A 70-year-old, Caucasian, ex-smoker man, presented with asthenia, anorexia, and weight loss of 5 kg during the last 2 months. Biological test showed high levels of transaminases and a microcytic, hypochromic anemia. Whole body CT-scan documented a gastric tumor lesion with concomitant loco-regional lymph node and hepatic metastases. Histology was consisted with the diagnosis of a primary GM. A double immunotherapy with nivolumab and ipilimumab was started but, 2 weeks later, the patient presented an acute hepatic failure quickly leading to his death despite a high dose corticotherapy. Conclusions: The particularity of this case relies on the rarity of GM, its difficult diagnosis representing a clinical challenge, and the complexity of its management that is not validated by large clinical trials, data being extrapolated from the treatment protocols routinely used in cutaneous melanoma. In our case, the patient died 2 weeks after the first cycle of a nivolumab/ipilimumab combined treatment for an acute hepatic failure that could be related to a treatment toxicity or a tumor hyperprogression. The patient's survival was very short not allowing any accurate evaluation of the efficacy of this therapy.

HER2-positive metastatic, parotid salivary duct carcinoma treated with a trastuzumab/pertuzumab-based chemotherapy: A case report.

This case highlights the rare entity, salivary duct carcinoma (SDC), which is difficult to diagnose and manage. It is the first published case of a metastatic, HER2-positive parotid SDC successfully treated by a dual anti-HER2 treatment associated to a chemotherapy.

Breast and Axillary Lymph Node Metastasis from Ovarian Cancer: A Case Report.

BACKGROUND Breast metastasis (BM) is extremely rare. Ovarian cancer accounts for approximately 0.03% to 0.6% of all BMs. BM diagnosis is challenging and the prognosis very poor. The treatment is multidisciplinary and strictly related to multiple clinical and biological factors. CASE REPORT A 70-year-old non-smoking Caucasian woman was hospitalized for a 4-month history of abdominal pain, anorexia, and weight loss of 10 kg. During the clinical examination, we found multiple axillary lymph nodes and a painless tumor lesion in the superior internal quadrant of the right breast. Whole body CT-scan and ¹8F-fluorodeoxyglucose PET scan documented a right ovarian tumor associated with multiple metastases, a hypermetabolic lesion of the right breast, and multiple axillary lymphadenopathies that were confirmed by breast ultrasonography. The percutaneous biopsy of both the right axillary lymph node and breast tumor showed a metastasis from a high-grade serous papillary ovarian adenocarcinoma. Considering the tumor aggressiveness and the lack of BRCA1 and BRCA2 mutations, we started systemic chemotherapy with a 3-week carboplatin/paclitaxel regimen combined with bevacizumab, which quickly improved the patient's symptoms and induced a biological tumor response. CONCLUSIONS This case reports a synchronous breast metastasis from an ovarian cancer and highlights this uncommon entity, which is very difficult to diagnose and treat. A differential diagnosis from a primary breast cancer should be considered as the treatment and prognosis of these 2 tumors are different.

Renal Metastases from a Nasal Cavity Mixed Squamous Cell and Adenoid Cystic Carcinoma: A Case Report.

BACKGROUND Adenoid cystic carcinoma (ACC) is a very rare tumor with a high risk of loco-regional recurrence and potential distant metastases. Until now, only a few cases of renal metastases from ACC have been reported in the literature. CASE REPORT A 64-year-old, Caucasian, non-smoker female, 8 months after being treated by radio-chemotherapy for a squamous cell nasal cavity tumor, presented two renal lesions associated with lung and vertebral metastases. Histology was consisted with a metastasis from an ACC. The histological revision of the primary nasal tumor confirmed a squamous cells carcinoma with an adenoid cystic component that metastasized to the kidney. Renal lesions appeared hypometabolic at the ¹8F-fluorodeoxyglucose (¹8F-FDG) PET scan mimicking a primary renal tumor. The patient underwent a systemic, palliative chemotherapy by a weekly carboplatin/paclitaxel/cetuximab regimen that was well tolerated and allowed a lasting tumor control. CONCLUSIONS The particularity of this case relies on the rarity of renal metastasis from ACC, its difficult diagnosis, and the complexity of its management, as no standard chemotherapy has been validated for metastatic ACC, yet. In our case, a weekly carboplatin/paclitaxel/cetuximab regimen was administered leading to a durable tumor stabilization with an excellent patient's quality of life.

A paraneoplastic limbic encephalitis from an anorectal small cell neuroendocrine carcinoma: a case report.

BACKGROUND: Paraneoplastic limbic encephalitis (PLE) is a rare autoimmune neurological syndrome observed in cancer patients. PLE is difficult to diagnose and presents a variable response to treatment, depending on the characteristics of the tumor and neuronal autoantibodies. CASE PRESENTATION: A 64-year-old, Caucasian, non-smoker man presented with a rapidly developing cognitive impairment, personality change, spatial disorientation, and short-term memory loss associated with anorexia and cervical and inguinal lymph nodes. The 18F-FDG PET scan documented intensely hypermetabolic lymph nodes, which histologically corresponded to a metastasis from a small cell neuroendocrine carcinoma. The brain MRI revealed a high T2-weighted FLAIR signal of the hippocamps, consisted with a PLE. The presence of anti-neuronal Hu antibodies confirmed the diagnosis. The patient underwent plasmapheresis, associated to a systemic chemotherapy resulting in a partial and temporary improvement of the neurological symptoms. Four cycles of intravenous immunoglobulins were also necessary. After six cures of chemotherapy, the lymph node metastases regressed. However, a new anorectal lesion was detected and was histologically confirmed as a primary small cell neuroendocrine carcinoma, which was treated with concomitant chemoradiotherapy. At the end of this treatment, the patient showed a rapid tumor progression leading to his death. CONCLUSIONS: This case highlights the rare entity, PLE, which is difficult to diagnose and manage. In addition, this is the first published case of PLE associated with an anorectal small cell neuroendocrine carcinoma, which appeared after completion of systemic chemotherapy.

A Spontaneous Regression of an Isolated Lymph Node Metastasis from a Primary Unknown Merkel Cell Carcinoma in a Patient with an Idiopathic Hyper-Eosinophilic Syndrome.

BACKGROUND Merkel cell carcinoma (MCC) is a rare, aggressive primary cutaneous neuroendocrine tumor frequently associated with Merkel cell polyomavirus infection. Despite its aggressiveness, a few reports of spontaneous MCC regression have been described in the literature, most of them following incisional biopsy supporting a hypothetical role of surgery-induced inflammation in the process of regression. CASE REPORT We report a case of 69-year-old Caucasian male who was followed for an idiopathic hyper-eosinophilic syndrome. A positron emission tomography (PET) scan documented a hyper-metabolic, left, inguinal adenopathy, histologically corresponding to a metastasis of a poorly differentiated neuroendocrine carcinoma. This lesion spontaneously regressed at clinical examination and radiological imaging. After its excisional dissection, histology was negative. Five months later, a nearby adenopathy reappeared. The patient underwent another excisional biopsy. Histology and immunohistochemistry were compatible with a lymph node metastasis of a MCC. As the patient refused radical surgery, a regional radiotherapy was performed. As of a follow-up at 10 months, he was alive and free of tumor recurrence. The hyper-eosinophilic syndrome was stable; however, the serum levels of chromogranin-A were inexplicably elevated in the absence of any tumor evidence at the PET scan. CONCLUSIONS The particularity of this case relies on the rarity of MCC complete spontaneous regression in a patient without a primary tumor and with a synchronous, idiopathic hyper-eosinophilic syndrome.

Peritoneal Myeloid Sarcoma in a Patient Treated for a Testicular Seminoma.

BACKGROUND Myeloid sarcoma is a rare extramedullary soft tissue neoplasm composed of myeloblastic cells, usually associated to hematologic tumor disorders and a poor prognosis. Its diagnosis is very difficult as radiological images are not specific. Histology and immunohistochemistry are necessary for an accurate diagnosis. CASE REPORT We report the case of 46-year-old, Caucasian, non-smoker male, treated in 2014 by orchiectomy and systemic chemotherapy for a stage IIB testicular seminoma. Considering the rapid increase of lactate dehydrogenase (LDH) levels without any evident medical reason, a computed tomography/positron emission tomography (CT/PET) scan was performed and revealing a diffuse, nodular, peritoneal tumor infiltration associated with multiple mesenteric and mediastinal adenopathies. Laparoscopy confirmed a diffuse tumor infiltration of the peritoneum. Histology and immunohistochemistry were consisted with the diagnosis of a myeloid monoblastic sarcoma. Cytology of bone marrow documented an monocytic acute myeloid leukemia. The patient started a systemic induction chemotherapy with high dose cytarabine and idarubicin that was complicated by an infectious pneumonia and colitis, and a grade IV thrombocytopenia leading to a brain subdural hemorrhage and quickly to patient's death. CONCLUSIONS We describe a rare, peritoneal, myeloid sarcoma in a young patient who had been treated by systemic chemotherapy for testicular seminoma 4 years earlier. The patient was clinically asymptomatic and presented only elevated LDH levels without any evident clinical reason. Considering the persistence of this biochemical abnormality, more investigations were performed leading to a diagnosis of peritoneal myeloid sarcoma associated with monocytic acute myeloid leukemia, probably secondary to the past chemotherapy.

Breast metastasis from squamous cell carcinoma of the oropharynx: a case report.

BACKGROUND: Breast metastases from extramammary tumors are extremely rare, the most common primary tumors being contralateral breast carcinoma, followed by lung, gynecological, gastrointestinal, melanoma, and hematological cancers. Only a few cases deriving from head and neck squamous cell carcinoma have been reported in the literature to date. CASE PRESENTATION: We report a case of a 47-year-old Caucasian woman who presented to our hospital with a solitary breast lesion in the right upper external quadrant associated with multiple bone and visceral metastases. Two years before, she had undergone radical resection of a squamous cell carcinoma of the oropharynx (stage pT2, pN1), which was followed by adjuvant radiotherapy. Breast ultrasound showed a hypoechogenic tumor lesion of 4 cm in the right upper external quadrant that was associated with multiple axillary and infra-/supraclavicular adenopathies. A positron emission tomographic scan documented multiple visceral and bone metastases with a single hypermetabolic lesion of the right breast. The results of histology and immunohistochemistry were consistent with a metastasis from a squamous cell carcinoma. The patient died of acute respiratory insufficiency 1 month after her breast metastasis diagnosis and before starting any systemic antitumoral treatment. CONCLUSIONS: Although breast metastases are extremely rare, they should be considered in any patient with a history of cancer and confirmed by histology and immunohistochemistry because they are very difficult to distinguish from other primary breast tumors based only on clinical and radiological features. There are no standardized treatment guidelines for breast metastasis management. Surgery and radiotherapy can play a role in symptom palliation, but they do not have any relevant impact on survival, the prognosis being poor, with an estimated overall survival less than 1 year from diagnosis.

Pituitary Metastasis from Renal Cell Carcinoma: Description of a Case Report.

BACKGROUND Pituitary metastasis is uncommon, breast and lung cancers being the most frequent primary tumors. Renal cell carcinoma (RCC) is a rare cause of pituitary metastases, with only a few cases described to date. CASE REPORT We report a case of a 61-year-old man who presented with a progressive deterioration of visual acuity and field associated with a bitemporal hemianopsia. Two years ago, he underwent radical right nephrectomy for a clear cell RCC (ccRCC). The biological tests showed pan-hypopituitarism and diabetes insipidus. Brain MRI revealed a large sellar tumor lesion bilaterally infiltrating the cavernous sinuses, which was surgically resected. Histology confirmed a ccRCC pituitary metastasis. The patient received post-surgical radiotherapy. Considering the presence of concomitant extra-pituitary metastases, treatment with sunitinib was started, followed by several lines of therapy with axitinib, everolimus, and sorafenib because of tumor progression. The patient also presented with a pituitary tumor recurrence, which was treated by stereotaxic radiotherapy. He died five years after the initial diagnosis of RCC and 30 months after the diagnosis of the pituitary metastasis.  CONCLUSIONS There are no standardized treatment guidelines for management of pituitary metastases. Pituitary surgery plays a role in symptom palliation, and it does not have any relevant impact on survival. Exclusive radiotherapy or stereotaxic radiotherapy could be an alternative to surgery in patients whose general condition is poor or who have concomitant extra-pituitary metastases.