A Case of Primary Signet-Ring Cell Cervical Carcinoma Treated with Chemoradiation, Brachytherapy, and Adjuvant Hysterectomy.

Primary signet-ring cell carcinoma of the uterine cervix is a rare subtype of cervical mucinous adenocarcinoma. Approximately 20 cases of primary signet-ring cell carcinoma of the cervix have been reported. Pathologic examination shows that adenocarcinomas with mucin accumulation in intracytoplasmic vacuoles displacing the nucleus indicate signet-ring cell carcinoma. A thorough metastatic workup is needed both for staging and to rule out gastrointestinal tract origin. Due to the rarity of the disease, both the true incidence and optimal management are unknown. Herein, the authors present a case of stage 1B3 primary signet-ring cell cervical carcinoma treated with combined chemotherapy and radiation (including external beam radiation and brachytherapy), followed by resection for residual disease. This case is consistent with limited reports where all surviving patients received surgery as well as 1 surviving patient with bulky disease required with chemoradiation and adjuvant hysterectomy.

Diagnosis of optic nerve sheath meningioma during optic nerve sheath decompression.

Optic nerve sheath meningioma (ONSM) is typically diagnosed based on clinical suspicion and imaging characteristics and is most often treated with radiation. Historically, biopsy, optic nerve sheath decompression, and debulking surgeries have been avoided for fear of optic nerve vascular disruption and tumor spread into the orbit. This is a case of a 48-year-old man who presented with unilateral optic disc edema, declining visual acuity, and a visual field defect. Despite an initial improvement with acetazolamide, his vision subsequently worsened. With an elevated lumbar puncture opening pressure and imaging showing right optic nerve sheath enhancement, the differential diagnosis included ONSM, perineuritis and idiopathic intracranial hypertension (IIH). Optic nerve sheath decompression (ONSD) with biopsy was performed, simultaneously decompressing the nerve and yielding a sample for pathologic analysis. A pathologic diagnosis of ONSM was made and treatment with radiation was subsequently initiated, but vision began to improve after the surgical decompression alone.

Regression of a paraganglioma tumor of the orbit.

PURPOSE: To describe a clinical case of an orbital paraganglioma that displayed regression after biopsy alone. METHODS: Case report. RESULTS: A 75-year-old female was examined for a right orbital tumor suspected to be metastatic breast carcinoma. An orbital biopsy was performed with significant hemorrhage encountered requiring extensive cautery. There was apparent clinical regression of the tumor with no signs of proptosis or eye movement restriction two years after this patient's biopsy. Histology was consistent with paraganglioma (glomus tumor). CONCLUSION: Although we cannot rule out spontaneous regression of this unique tumor, we postulate that tissue necrosis caused by the use of cautery induced regression. Unless encapsulated and easily accessible, we suggest that the best management of this rare tumor is that of observation after being found negative for malignancy by biopsy given their propensity for slow progression and in rare cases, regression.

Juvenile granulosa cell tumor of the testis.

Juvenile granulosa cell tumor of the testis is a rare type of intermediate stromal cell tumor. Only 11 cases of juvenile granulosa cell tumor of the testis have been reported to the national tumor registry as of December 2001. We report an additional case of primary juvenile granulosa cell tumor of the testis. The benign lesion was managed successfully with orchiectomy, and follow-up computed tomography revealed no evidence of recurrence at 6 months. The histopathologic features, treatment, and prognosis of this unusual tumor are reviewed.