An unusual case of hydranencephaly presenting with an anterior midline cyst, a posterior calcified mass, cerebellar hypoplasia and occlusion of the posterior cerebral arteries.

We present an unusual case of severe hydranencephaly in a term infant who presented with the following additional unique features, which were discovered on CT, MRI and MR angiography examinations: (1) occlusion of the bilateral posterior cerebral arteries, (2) absence of the occipital lobes, (3) an ovoid calcified mass sitting on the inner table of the occipital bone, (4) severe cerebellar hypoplasia, (5) a dysmorphic cystic diencephalon, (6) a large anterior midline cyst just above the cribriform plate and (7) absence of the falx. These imaging findings were confirmed at autopsy.

Squamous cell carcinoma arising in a second branchial cleft cyst.

The existence of primary branchiogenic carcinoma is controversial. In 1950, Martin et al. established four criteria for the diagnosis of primary branchiogenic carcinoma. In 1989, Khafif et al. proposed new criteria, which are currently most recognized in the literature. A pathologic description of the surgical specimen is analyzed and compared with a critical review of the literature. A case is presented in which imaging established the diagnosis of a benign second branchial cleft cyst 2 years prior to the diagnosis of a branchiogenic carcinoma in the same cyst. This cyst was resected and was found to have squamous cell carcinoma arising from the benign epithelium. This case satisfies the histologic criteria established by Martin and Khafif for a primary branchiogenic carcinoma arising in a previously benign second branchial cleft cyst.