1. Abstract A 23 years old girl, without history, consulted for a neck tumefaction appeared 20 days ago, which spread then to the abdomen and lower limbs with Asthenia, dysphagia, abdominal pain, effort dyspnea. Clinical examination revealed an altered general state, obesity with centripetal fat deposition face, supraclavicular and dorsal and cervical fat pads, facial plethora, rounded face, buffalo-hump, face acne, melanodermia, capillary fragility, face, arms and legs hirsutism.
Keywords: Small Cell Lung Cancer; Neuroendocrine Tumor; Pediatric; Lung Cancer; Cortisol; Paranesoplasic; Cushing Syndrome
2. Introduction Adolescent Small Cell Carcinoma is a rare entity. The first case was reported with a 14-year- old boy. It presented 0.2 % of children tumors. These neuroendocrine tumors are associated in 1 to 5% of cases to a paraneoplastic Cushing's syndrome. The second paraneoplastic syndrome in order of frequency is the syndrome of inappropriate secretion of ADH. Observation A 23 years old girl, without history, consulted for a neck tumefaction appeared 20 days ago, which spread then to the abdomen and lower limbs with Asthenia, dysphagia, abdominal pain, effort dys pnea. Clinical examination revealed an altered general state, obesity with centripetal fat deposition face, supraclavicular and dorsal and cervical fat pads, facial plethora, rounded face, buffalo-hump, face acne, melanodermia, capillary fragility, face, arms and legs hirsutism (Figure 1-4).
3. Discussion Primary lung cancers are rare in children [1]. It presents 0.16% of all lung cancers occurring in the first decade of life and 0.7% in the second decade [2]. Reported cases of pediatric lung carcinoma in literature are most commonly undifferentiated carcinoma, followed by adenocarcinoma and squamous cell carcinoma. There was some historical cases of undifferentiated carcinoma truly represent small cell carcinoma or perhaps atypical carcinoid tumors in literature. In pediatric population, most common benign lung tumor is inflammatory myofibroblastic tumor (52%), and the most common cancer are carcinoid tumor and pleuropulmonary blastoma [3]. Trachea, bronchus, and lungs cancers represent 0.2% of all children cancers [4]. Eighteen to thirty years old patients with lung cancer have a high incidence of female sex, there is no association with smoking, and favorable prognosis [5]. In 2000, Kim et al published the first case of a 14-year-old boy with Small cell lung cancer. Surveillance Epidemiology and End Results review during twenty one years reported 7 cases of pediatric small cell lung cancer, when Children’s Hospital Boston found 1 case whereas a 90-year review [6-12]. They had the worst prognosis in pediatric lung cancer with median survival less than 5 months. The overall survival was generally poor, and the majority dies with disease recurrence. Two-year survival in patients with extensive stage was 4.6%, and five-year survival of patients with limited stage was 10%. First-line chemotherapy was four to six cycles of Etposide-Cisplatin. Eighty per cent of limited stage patients and all extensive patients relapse within the first year after initial treatment. In recurrent disease, median survival was 2 to 3 months. A literature review by PubMed was done by using search terms “small cell lung cancer” and “neuroendocrine carcinoma lung,” and “neuroendocrine tumor lung.” The search was limited to articles describing patients 14 to 18 years old. Six cases were reported (Table 1).
4. Conclusion Small cell cancer, high grade neuroendocrine lung cancer is extremely rarein adolescent population. Few cases were reported in literature, rarer those associated to paraneoplastic syndrome. Patients with an extensive stage tumor have a bad prognosis with 2 year survival less the 5 %.
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Mnif Ahmed. Small Cell Carcinoma with Paraneoplastic Cushing’s Syndrome in A 23 -Year-Old A New Observation and Literature Review. Annals of Clinical and Medical Case Reports 2021