Faculty Bibliography

Intracranial disease is a very rare presentation at diagnosis in acute promyelocytic leukemia (APL). The risk associated with this particular presentation is not accounted for when the current risk stratification is based on peripheral counts. Extra medullary disease in general may challenge this risk stratification that commands initial induction treatment of this potentially fatal disease. Here we discuss a case presented at diagnosis with extensive intracranial base of the skull, clivus and sinus infiltration and heavily infiltrated bone marrow yet with low peripheral blood counts and no peripheral blood blasts. Such cases lack evidence of how to treat.

Case Presentation: We describe three cases of non-HPV-associated gastric-type mucinous endocervical adenocarcinoma in patients (ages 24, 28, and 58 years) at NYU-Winthrop. All patients presented with high-stage tumors; two patients were treated with pelvic exenteration and long-term chemotherapy, and one is receiving neoadjuvant chemotherapy. Although these are aggressive tumors, all patients are currently clinically stable. Literature Review: In the WHO 2014 classification, gastric- type mucinous endocervical adenocarcinoma (GAC) of the cervix was classified as a subtype of cervical mucinous carcinoma. Clinically, patients range from 32 to 84 years and may be asymptomatic or may present with vaginal bleeding. The cervix may be enlarged and indurated or may be normal. Often, routine Pap smears are normal, with negative HPV studies. On histologic examination, this tumor has distinctive features-abundant glandular epithelium comprising mucin-rich columnar cells, with bland basal nuclei, no significant mitotic activity, and voluminous clear cytoplasm with foamy cells. No in situ component is identified. The tumor cells are negative for p16 and HPV but contain gastric-type mucin (HIK 1083). Clinical Significance and
Conclusion(s): GAC is an aggressive tumor, often seen in young patients, characterized by advanced stage at presentation and an aggressive clinical course with unusual patterns of spread, with a propensity for peritoneal, omental, and adnexal dissemination. The 5-year disease-free survival for GAC is around 42% and about 91% for usual type endocervical adenocarcinoma. Routine Pap smears and HPV screening are not useful. With large-scale HPV vaccination programs under way, the prevalence of non-HPV-positive tumors such as GAC will increase in proportion. Therefore, awareness of the lesion, a high index of suspicion in the correct clinical context, and pathologic recognition of this morphologically subtle lesion are mandatory