Pancreatic metastasis of a pleuropulmonary blastoma in an adult

Abstract

SummaryPleuropulmonary blastoma (PPB) is a rare dysontogenetic tumor that usually develops in the first decade of life and has been recognized as a distinct clinico-pathological entity different from the ordinary pulmonary blastoma of adulthood. Since the tumor grows aggressively and tends to metastasize early, physicians have to be aware of late onset of symptoms and uncommon manifestations. We report a case of PPB in a young adult and its recurrence in the pancreas after primary surgical treatment and adjuvant chemotherapy. Keeping in mind the moderate prognosis of PPB in children, accurate assessment and treatment of PPB require a team approach of oncology, radiology and surgery to establish new therapeutic guidelines in the future.

Pleuropulmonary blastoma in an adult: A case report

“…Often presenting symptoms are fever, cough, hemoptysis, dyspnea and chest pain. The clinical picture of PPB in an adult resembles that in children [5]. In our case also, the patient presented with cough, dyspnea and fever.…”

DICER1 syndrome: Approach to testing and management at a large pediatric tertiary care center

“…In addition, a small number of cases have been identified on prenatal ultrasound . Two cases of type 2 PPB have been reported at ages 21 and 36 and a type 3 has been reported at age 18 . Imaging scenarios for type 1 PPB, employing alternating chest radiographs and chest CT, suggested decreased loss of life expectancy due to undetected disease .…”

A Pleuropulmonary Blastoma: A Rare Malignant Lung Disease in Children

“…3 It occurs in an exclusive manner in children and mostly involves local evolution with some cases presenting as metastasizing and aggressive tumors. 4 While PB in adults is more common than children's type of (PPB), PB occurs commonly in the middle-aged and is typically associated with symptoms and signs similar to lung cancer. It is divided into two groups depending on histopathological features: type (1) monophasic PB, which is called well-differentiated fetal adenocarcinoma (WDFA), and type (2), which is classic biphasic PB (CBPB) containing tissue of both fetal adenocarcinoma (type 2 is typically low grade) and primitive mesenchymal stroma.…”