Small cell carcinoma of the breast: case report.

BACKGROUND: Small cell neuroendocrine carcinoma of the breast is a rare tumor with fewer than 30 cases reported in the literature. The reported age of incidence of mammary small cell carcinoma is similar to that of breast carcinoma of the usual types. CASE: The clinicopathologic findings of a primary mammary small cell neuroendocrine carcinoma occurring in a 28-year-old female are presented with a review of pertinent literature. She was treated with lumpectomy and sentinel node biopsy as well as chemotherapy and radiotherapy. CONCLUSIONS: To the best of our knowledge, this is the youngest patient with primary small cell carcinoma of the breast reported in the English literature, indicating that these tumors occur in a wide age range.

Ovarian ossification associated with endometriosis.

BACKGROUND: Bone formation in the ovary, with the exception of developing in the setting of mature cystic teratoma, is exceedingly rare. CASE: A 46-year-old woman with a history of endometriosis and chronic pelvic pain underwent total abdominal hysterectomy and bilateral salpingo-oophorectomy. A 3 cm solid heavily calcified mass with a stony hard consistency was detected within the right ovary. Microscopic examination revealed extensive calcification of the right ovarian stroma with formation of abundant mature bone, adjacent to small foci of endometriosis. CONCLUSIONS: Endometriosis can be associated with ovarian ossification, forming an extensively calcified adnexal mass. Conservative treatment with close follow-up may be adequate in patients with a history of endometriosis who present with a small heavily calcified ovarian mass and wish to preserve their fertility.

[Placental site trophoblastic tumor].

Placental site trophoblastic tumor (PSTT) is a rare form of gestational trophoblastic disease (GTD) that originates from the implantation site intermediate trophoblast. It accounts for about 1% of all GTDs, with an estimated incidence of 1 per 100,000 pregnancies. Most patients are in their thirties and the prevailing presenting symptom is abnormal vaginal bleeding. More than half of the patients present with disease limited to the uterus and the remainder present with disease extension beyond the uterus. The overall mortality rate is 25%. The most important adverse prognostic factor is disease extension beyond the uterus. Other adverse prognostic factors are interval from antecedent pregnancy > 2 years, mitotic count > 5 mitotic figures/10 high-power fields, and age > 40 years. Since PSTT is less sensitive to chemotherapy than GTDs originating from cytotrophoblast and syncytiotrophoblast (hydatidiform mole, invasive mole, and choriocarcinoma), hysterectomy is the mainstay of treatment. Systemic multi-agent chemotherapy is administered in the presence of disease extension beyond the uterus and considered in the presence of other adverse prognostic factors. The EP/EMA regimen seems to be the most effective chemotherapy available to date for PSTT. Although PSTT produces less human chorionic gonadotropin (hCG) than choriocarcinoma, beta-hCG is still the best available serum marker for monitoring the response to treatment and for follow-up.

Pedunculated aggressive angiomyxoma arising from the vaginal suburethral area: case report and review of literature.

BACKGROUND: Aggressive angiomyxoma (AA) is an uncommon, slow growing, locally infiltrative but non-metastasizing, distinctive mesenchymal tumor that predominantly affects the pelvis and perineum of premenopausal women. The mainstay of treatment is local excision with tumor-free margins; however, recurrences are common and related to inadequate primary excision. CASE: A pedunculated 3-cm mass arising from the vaginal suburethral area in a 49-year-old premenopausal woman was resected around the base of its pedicle. Microscopic examination revealed numerous blood vessels of various sizes set in myxoid stroma with spindle shaped fibroblasts. Immunohistochemical staining was strongly diffusely positive for vimentin, desmin, estrogen receptor (ER) and progesterone receptor (PR), weakly focally positive for CD34, and negative for S-100 protein, actin and Ki-67. These findings are compatible with the diagnosis of AA. To date, six months after surgery, the patient is alive and without evidence of recurrence. CONCLUSIONS: AA is often clinically misdiagnosed and it is only the microscopic examination strengthened with immunohistochemical staining that definitely and undeniably contributes to the final diagnosis of AA. Based on this case report and on the previously reported five cases of pedunculated AA arising from the vulvovaginal region, including one tumor arising from the vaginal suburethral area, it seems that pedunculated AAs arising from the vulvovaginal region are at negligible risk of recurrence after local excision.

Primary peritoneal malignant mixed müllerian tumor associated with colonic adenocarcinoma.

BACKGROUND: Primary extragenital malignant mixed Müllerian tumors (MMMTs) are very rare neoplasms, with only 28 documented cases in the literature. Two cases coexisted with a colonic adenocarcinoma. CASE: We report on a primary peritoneal MMMT diagnosed shortly after resection of a colonic adenocarcinoma in an 85-year-old woman who presented with a large omental mass. Microscopic examination revealed a biphasic tumor with malignant carcinomatous and sarcomatous components, confirmed immunohistochemically, consistent with MMMT. Despite optimal debulking and uneventful postoperative recovery, the patient died of her disease shortly after surgery due to recurrent disseminated disease. CONCLUSIONS: This is the third case in the literature of primary extragenital MMMT occurring in association with colonic adenocarcinoma. This coexistence may be incidental, but it may also imply a possible linkage between these two tumors.

Vulvar invasive squamous cell carcinoma occurring in a young woman with systemic lupus erythematosus.

BACKGROUND: Although several studies have demonstrated a possible relationship between systemic lupus erythematosus (SLE) and non-Hodgkin's lymphoma, Hodgkin's lymphoma, leukemia and several solid tumors, it is still debatable whether SLE patients have an increased incidence of cancer overall. CASE: We describe a 25-year-old patient with SLE who developed invasive squamous cell carcinoma of the vulva. The patient underwent radical vulvectomy and bilateral groin sentinel lymph node dissection and until to date, one year after surgery, she is alive without evidence of recurrent disease. CONCLUSIONS: Only three cases of vaginal/vulvar cancer associated with SLE have previously been mentioned in the literature, but not described in detail. This is the first detailed case report in the literature of vulvar invasive squamous cell carcinoma occurring in a SLE patient. It can only be speculated that the SLE itself and/or the treatment with immunosuppressive drugs provoked malignant transformation and the development of vulvar squamous cell carcinoma in such a young patient.

Alpha-fetoprotein production by pancreatic tumors exhibiting acinar cell differentiation: study of five cases, one arising in a mediastinal teratoma.

Five cases of pancreatic neoplasms accompanied by production of alpha-fetoprotein (AFP) and serum elevation of this marker are presented, and the better-documented cases of this phenomenon from the literature are reviewed. Four of the cases originated in the orthotopic pancreas, whereas the fifth arose in the pancreatic component of a mediastinal teratoma. The patients were children or young adults. AFP production in pancreatic tumors is closely linked to acinar differentiation, most cases representing either pancreatoblastoma or acinar cell carcinoma. The distinction between these 2 tumors may be difficult because of the many morphologic and immunohistochemical features they share.

Placental site trophoblastic tumor: report of four cases and review of literature.

Placental site trophoblastic tumor (PSTT) is a rare variant of gestational trophoblastic disease that originates from the implantation site intermediate trophoblast. We report four patients with PSTT and review pertinent literature. Three patients presented with disease confined to the uterus and one patient with disease extension beyond the uterus. Antecedent pregnancy was full-term pregnancy in three patients and termination of a 21-week pregnancy in one patient. Interval from the antecedent pregnancy was <1 year in three patients and 13 years in one patient. Primary treatment was simple hysterectomy in three patients and radical hysterectomy in one patient. Overall, three patients received chemotherapy; one had EP/EMA as adjuvant chemotherapy, one had EMA/CO for rising levels of serum beta-hCG and one had BEP then VIP for recurrent disease. The three patients with disease confined to the uterus have remained after treatment alive and with no evidence of disease, whereas the one patient with disease extension beyond the uterus died of disease despite surgery and aggressive chemotherapy. It is concluded that disease extension beyond the uterus is the most important adverse prognostic factor. Other adverse prognostic factors are interval from antecedent pregnancy >2 years, age >40 years, and mitotic count >5 mitotic figures/10 high-power fields. Because of the relative insensitivity to chemotherapy, hysterectomy is the mainstay of treatment. EP/EMA seems to be the most effective first-line chemotherapy available to date for metastatic and relapsing PSTT.

Mucinous cystadenoma of the ascending colon: a novel presentation.

Mucinous cystadenomas usually occur in the appendix, ovaries and pancreas. This is the first report to describe mucinous cystadenoma of the ascending colon. A 32-year-old female presented with abdominal pain. Radiographic studies demonstrated a space-occupying lesion in the ascending colon associated with intussusception. Right colectomy revealed a cystic mass within the ascending colon that contained thick mucin and was lined by mucin-producing columnar epithelium. Clinical and pathologic features with a brief review of the literature are presented.

Congenital epulis.

Epulis is a rare tumor of the newborn, also known as granular cell tumor of the newborn or Neumann's tumor. This tumor arises from the mucosa of the gingiva, most commonly from the anterior part of the maxillary alveolar ridge, and is typically seen as a mass protruding out of the newborn child's mouth, which may interfere with respiration or feeding. Epulis is seen only in the newborn and is a different entity from other granular cell tumors. The tumor has a marked female preponderance of 8:1. The recommended treatment is prompt surgical resection. Recurrences of the tumor and damage to future dentition have not been reported, suggesting that radical excision is not warranted. A newborn female with such a mass is described. The tumor was resected using a carbon dioxide laser; the postoperative course was uneventful. On histologic examination, it was composed of diffuse sheets and clusters of polygonal cells containing small round to oval nuclei and abundant coarsely granular cytoplasm. The tumor cells stained positive for vimentin, and negative for S100-protein, actin, desmin, laminin, keratin, estrogen, and progesterone receptors. Electron microscopic examination showed granular cells containing heterogeneous electron-dense granules, lysosomes, and cytoplasmic lipid droplets. The clinical and microscopic features of such tumors are reviewed.