The Impact of Radiation on an Unusual Case of Omental Epithelioid Angiosarcoma.

Epithelioid angiosarcoma is a rare high-grade tumor with irregular vascular morphology. We report an unusual case of intra-abdominal epithelioid angiosarcoma affecting the omentum and peritoneal surfaces resulting in significant hemorrhagic and inflammatory changes. As in other cases of this tumor this patient had previously undergone radiation treatment for a history of cervical cancer.

Primary and metastatic parathyroid malignancies: a rare or underdiagnosed condition?

OBJECTIVE: Parathyroid gland malignancies are considered rare. The most common of these tumor types is primary parathyroid carcinoma. Metastatic spread from other cancers may also occur with up to 10% of cancers from other sites showing parathyroid involvement at autopsy. Tumor-to-tumor metastases (metastatic spread to parathyroid neoplasm) from remote cancers to the parathyroid gland have been described. METHODS: We did a PubMed literature review and analysis of our own experience of 392 consecutive parathyroidectomies. RESULTS: Primary and secondary parathyroid malignancies can be grouped into three categories: primary parathyroid carcinoma (PPCa), spread of carcinoma into parathyroid glands by contiguous extension from the thyroid gland or other head and neck cancer, and metastatic disease to the parathyroid gland from distant cancers. Studies of tumor-to-tumor spread indicate a predilection of spread to endocrine tumors possibly because of the rich blood supply that is present in endocrine tumors. Two of our 392 parathyroidectomies (0.5%) had cancer: one metastatic (thymic neuroendocrine tumor) and another PPCa. CONCLUSION: Metastatic disease to the parathyroid gland is poorly documented. When performing surgery for primary thyroid cancer, the search for parathyroid gland metastases is often overlooked because of the desire to preserve parathyroid function. Metastatic disease from other cancers to a benign parathyroid gland or to a parathyroid adenoma probably suggests a grave prognosis because it likely indicates widespread metastatic disease; however, isolated metastases to the parathyroid may occur. Although these lesions may be uncommon they may not be as rare as once thought.

Pregnancy following Radical Resection of Solid Pseudopapillary Tumor of the Pancreas.

Solid pseudopapillary tumor of the pancreas is a rare tumor seen in predominately young women and carries a low malignant potential. We discuss a patient, who presented to our high risk clinic, with a clinical history of solid pseudopapillary tumor of the pancreas, predating her pregnancy. The patient had undergone previous surgery and imaging which had excluded recurrence of disease; however, increased attention was paid to the patient during her pregnancy secondary to elevated hormonal levels of progesterone, which any residual disease would have a heightened sensitivity to. In cases of pregnant patients with a history of pancreatic tumors, a multidisciplinary approach with maternal fetal medicine, medicine, and general surgery is appropriate and can result in a healthy mother and healthy term infant.

Idiopathic granulomatous mastitis with erythema nodosum simulating breast abscess in pregnancy: A case report.

Granulomatous mastitis is a rare benign inflammatory condition of the breast and is known to be associated with pregnancy. A 25-year-old Hispanic G2P1 at 17 weeks gestation presented to the emergency department with findings consistent of a breast abscess. The abscess failed to resolve with incision and drainage followed by multiple courses of antibiotic therapy. A biopsy was then obtained and yielded a diagnosis of granulomatous lobulitis. The patient was treated with steroids and her symptoms resolved. Granulomatous lobulitis may present with characteristics of various clinical entities including neoplasm or, as in this case, abscess. Clinicians should consider a diagnosis of granulomatous mastitis in cases of recalcitrant breast abscess.

Neuroendocrine thymic carcinoma metastatic to the parathyroid gland that was reimplanted into the forearm in patient with multiple endocrine neoplasia type 1 syndrome: a challenging management dilemma.

OBJECTIVE: To describe a unique case of a metastatic thymic carcinoma to the hyperplastic parathyroid gland and to present a challenging management dilemma. METHODS: Our patient is 60-year-old, intellectually disabled man with history of the multiple endocrine neoplasia type 1 (MEN1) syndrome, a surgery in 1985 for hypercalcemia with removal of one parathyroid gland, surgery in 2007 with findings of extensively necrotic well differentiated neuroendocrine carcinoma (carcinoid tumor) of the thymus. In 2012, he presented with persistent hypercalcemia (calcium level 11.7 mg/dL [range, 8.6-10.2]), and a parathyroid hormone (PTH) level of 225 pg/mL (range, 15-65 pg/mL). He underwent a repeat neck exploration with removal of 2 small inferior and a large left superior 4.5 × 2.5 × 1.5 cm parathyroid glands, all of which showed hyperplasia on intraoperative frozen section. A small portion of the superior gland was reimplanted into the patient's forearm. Final pathology showed the presence of a focus of neuroendocrine tumor within the left superior parathyroid gland with immunostain identical to the thymic carcinoma. His postoperative PTH level was 14 pg/mL and calcium 8.5 mg/dL. A positron emission tomography-computed tomography (PET-CT) and octreotide scans revealed an extensive metastatic disease within the lung, mediastinum, and bones. RESULTS: We decided to leave a portion of the reimplanted parathyroid gland with possible metastatic thymic carcinoid in his forearm because of the presence a widespread metastatic disease and his intellectual disability that would result in noncompliance with calcium replacement in case of permanent hypocalcemia. CONCLUSION: Metastatic thymic carcinoma to the parathyroid gland has never been reported in the literature. We have described the first case and presented a challenging management dilemma.

CD117, Ki-67, and p53 predict survival in neuroendocrine carcinomas, but not within the subgroup of small cell lung carcinoma.

High-grade neuroendocrine carcinomas (NECs) are aggressive tumors with limited treatment options. Recently, studies have observed that the tyrosine kinase receptor CD117 is often overexpressed in this malignancy. As a result, CD117 has been identified as a target for therapy via the small molecule, tyrosine kinase inhibitor imatinib mesylate. In the present study, 17 low-grade, 4 intermediate-grade, and 76 high-grade NECs were immunostained for CD117, Ki-67, and p53. Overexpression of the three markers was mainly, but not exclusively seen in the high-grade NECs. Patients with overexpression of CD117 and p53 and increased Ki-67 expression showed reduced survival. However, no difference in survival was observed when the same analysis was applied solely to small cell lung cancer patients, the largest subset studied. These findings suggest that overexpression of CD117, p53, and Ki-67 reflects tumor grade and predicts survival in NECs, but fail as prognostic markers in the subset of small cell lung cancer patients.

Single nucleotide polymorphisms act as modifiers and correlate with the development of medullary and simultaneous medullary/papillary thyroid carcinomas in 2 large, non-related families with the RET V804M proto-oncogene mutation.

BACKGROUND: Single nucleotide polymorphisms (SNPs) may function as modifiers of the RET proto-oncogene, resulting in the expression of medullary thyroid carcinoma (MTC) and papillary thyroid carcinoma (PTC). We present 2 non-related Italian-American families (Family 1, n = 107; Family 2, n = 31) with the RET V804M mutation. We have correlated the presence of specific SNPs and the rare RET V804M mutation to MTC, C-cell hyperplasia (CCH), and PTC. METHODS: Sequencing was performed on exons 10, 11, and 13-16 of the RET proto-oncogene. The presence of MTC, CCH, and PTC were correlated to specific SNPs. RESULTS: In both families, 3 SNPs in exon 11 (G691S), exon 13 (L769L), and exon 15 (S904S) were detected in 100% of patients with overt MTC. The SNP L769L was present in all patients including patients with PTC, MTC, and CCH. CONCLUSION: SNP analysis revealed a similar pattern between the 2 families. SNPs in exon 11 (G691S) and exon 15 (S904S) appear to influence the development of MTC. A SNP in exon 13 (L769L) may serve as a modifier in the development of simultaneous MTC and PTC, as well as presentation of MTC, in patients with the RET V804M mutation.

One hundred and seven family members with the rearranged during transfection V804M proto-oncogene mutation presenting with simultaneous medullary and papillary thyroid carcinomas, rare primary hyperparathyroidism, and no pheochromocytomas: is this a new syndrome--MEN 2C?

BACKGROUND: The rearranged during transfection (RET) V804M proto-oncogene mutation is rare and associated with medullary thyroid carcinoma (MTC). We present 40 members from a total cohort of 107 family members with this mutation. METHODS: Family members were tested for RET mutations, calcitonin levels, and screened for pheochromocytoma and primary hyperparathyroidism (PHPT). Thyroidectomies were performed on 15 members. Surgery and pathology reports were obtained and reviewed. A pedigree was constructed. RESULTS: A high penetrance was found for MTC and simultaneous papillary thyroid carcinoma (PTC; 40%). The incidence of PHPT was low (13%). There were no findings of pheochromocytoma. The course in the first family generation was indolent, with late onset of MTC. The second generation experienced earlier disease development; onset occurred earliest in the third generation. The second generation experienced a higher incidence of PTC than the first. CONCLUSION: This is the largest family with this mutation reported to date. However, it does not fit the classic familial MTC or MEN 2A cancer syndrome. Considering that PTC is not an incidental finding, but the result of an inherited RET V804 M mutation, we propose to identify this phenotypic expression as a unique syndrome consistent with manifestations of MTC, PHPT, and PTC.

Use of hormone suppression then oocyte freezing to preserve reproductive capability in an adolescent girl with ovarian luteinized thecoma associated with sclerosing peritonitis.

OBJECTIVE: To describe a case of ovarian luteinized thecoma, a rare ovarian neoplasm, which is only the 26th reported case associated with sclerosing peritonitis. DESIGN: Case report. SETTING: NYU Fertility Center and Memorial Hospital for Cancer and Allied Diseases, New York. PATIENT(S): A 17-year-old woman presenting with a large pelvic mass and abdominal pain. INTERVENTION(S): Conservative surgical treatment with laparotomy, unilateral salpingooophorectomy, and biopsy of contralateral ovary. Gonadotropin-releasing hormone agonist suppression. Ovarian hyperstimulation with oocyte retrieval/freezing to preserve biologic fertility. MAIN OUTCOME MEASURE(S): Response to conservative therapy and oocyte cryopreservation as a method of fertility preservation. RESULT(S): At laparotomy, obvious unilateral ovarian involvement was present, and a left salpingoophorectomy was performed. Biopsy of the contralateral ovary confirmed bilateral disease. The initial pathological review was complicated by extensive ovarian edema. The patient was treated with gonadotropin-releasing hormone agonist suppression plus intermittent estradiol supplementation. When she became intolerant of hormone therapy and when removal of the remaining ovary became a possibility, she underwent ovarian hyperstimulation; oocyte retrieval and freezing were performed to preserve her biologic fertility. Thirty-eight eggs were obtained. CONCLUSION(S): Surgically diagnosed luteinized thecoma can be managed medically. Oocyte cryopreservation as a means of fertility preservation should be considered in young women with this diagnosis who are at risk for bilateral gonad removal.