Plasma cell leukemia (PCL) is an aggressive form of multiple myeloma where there is hematogenous spread of abnormal plasma cells into the periphery. This is opposed to multiple myeloma, where the abnormal plasma cells stay in the bone marrow. PCL is more common in males than females, and is also more common in African-Americans than Caucasians. Signs and symptoms of PCL include, but are not limited to, renal insufficiency, hypercalcemia, anemia, lytic bone lesions, thrombocytopenia, hepatomegaly, and splenomegaly. Here, we discussed a case of a 71-year-old Caucasian female recently diagnosed with primary PCL with radiographic features of this disease throughout the body, with an emphasis on the maxillofacial skeleton and relevance from a dental standpoint.
Aged ; Anemia ; Bone Marrow ; Female ; Hepatomegaly ; Humans ; Hypercalcemia ; Leukemia, Plasma Cell* ; Male ; Maxilla* ; Multiple Myeloma ; Pathology ; Plasma Cells* ; Plasma* ; Renal Insufficiency ; Skeleton ; Splenomegaly ; Thrombocytopenia

OBJECTIVETo study the clinicopathologic features of small cell carcinoma of ovary, hypercalcemic type (SCCOHT) and to evaluate the diagnostic significance of loss of SMARCA4 expression.

METHODSThe clinicopathologic characteristics of 5 cases of SCCOHT were reviewed. The expression of SMARCA4 protein was detected by immunohistochemistry in the cases of SCCOHT and 240 cases of other primary malignant tumors of ovary and peritoneum.

RESULTSThe mean and medium age of these patients was 30 years and 28 years, respectively. The presenting symptoms included abdominal pain, distention and a pelvic mass. Hypercalcemia was found in 3 patients. The maximum diameter of tumors ranged from 13.5 to 22.0 cm. Extraovarian spread was demonstrated in all of the patients on presentation. Histologically, the tumors were composed of closely packed small round cells with scanty cytoplasm, hyperchromatic nuclei and irregular chromatin clumps. The tumor cells grew in sheets, nests, cords or trabecular pattern. Follicle-like spaces were observed in 4 cases. Three of the tumors contained large cells with abundant eosinophilic cytoplasm. Spindle cell morphology was found in 1 case. There were 2 cases with myxoid or hyaline stroma. Four out of five of SCCOHT cases showed loss of SMARCA4 protein while only 6.3% (15/240) of the other primary malignant tumors of ovary and peritoneum , including undifferentiated carcinoma (1/5), high-grade serous carcinoma (4.6%, 5/109), endometrioid carcinoma (7.7%, 2/26), clear cell carcinoma (1/9), mucinous carcinoma (1/5), mixed carcinoma (4.9%, 3/61), carcinosarcoma (1/9) and high-grade serous carcinoma of peritoneum (1/9), were negative.

CONCLUSIONSSCCOHT is a rare malignant tumor and often misdiagnosed as other types of ovarian small cell tumor. Loss expression of SMARCA4 protein is characteristic and facilitates the diagnosis and differential diagnosis of SCCOHT.

Adenocarcinoma, Mucinous ; Adult ; Carcinoma, Small Cell ; genetics ; metabolism ; pathology ; DNA Helicases ; genetics ; metabolism ; Female ; Humans ; Hypercalcemia ; pathology ; Immunohistochemistry ; Neoplasms, Glandular and Epithelial ; genetics ; metabolism ; pathology ; Nuclear Proteins ; genetics ; metabolism ; Ovarian Neoplasms ; genetics ; metabolism ; pathology ; Transcription Factors ; genetics ; metabolism

Paraneoplastic hypercalcemia without bone metastasis occurs rarely in esophageal cancer. A 75-year-old man was admitted for general weakness and lethargy. Laboratory data showed high serum calcium level (corrected calcium 14.6 mg/dL), low parathyroid hormone level (3.3 pg/mL) and high parathyroid hormone-related peptide level (3.5 pmol/L). Esophagogastroscopy showed a malignant tumor in the esophagus. Histology showed moderately differentiated squamous cell carcinoma. Bone scan showed no evidence of bone metastasis. Since the patient's calcium levels remained high and mental state did not show improvement despite intravenous fluid therapy, diuretics and intravenous bisphosphonate, hemodialysis was started. After hemodialysis treatment, the serum calcium level subsequently normalized and his mental status improved. Herein, we report a rare case of paraneoplastic hypercalcemia in a patient with esophageal cancer.
Aged ; Calcium/blood ; Carcinoma, Squamous Cell/*diagnosis/pathology ; Endoscopy, Digestive System ; Esophageal Neoplasms/*diagnosis/pathology ; Humans ; Hypercalcemia/*diagnosis/pathology ; Male ; Parathyroid Hormone-Related Protein/blood ; Renal Dialysis ; Severity of Illness Index ; Tomography, X-Ray Computed

OBJECTIVETo study the clinicopathologic characteristics of parathyroid carcinoma (PTC).

METHODSEleven cases of PTC encountered during the period from 1994 to 2012 were enrolled into the study. Forty cases of parathyroid adenoma (PA) were also retrieved for comparison. The clinical manifestations, laboratory results and pathologic features were analyzed, with literature review.

RESULTSThe main clinical manifestations of PTC included neck mass (11/11), hypercalcemia (11/11) and hyperparathyroidism (11/11). Most patients also had osteoporosis (10/11). In contrast, PA often manifested as hypercalcemia (40/40) and hyperparathyroidism (40/40). Histologic examination of PTC showed that the tumor cells contained clear to eosinophilic cytoplasm and separated by dense bands of fibrosis. The tumor mass was surrounded by thick fibrous capsule. Foci of capsular invasion and vascular permeation were identified at the tumor periphery in all cases. Cellular atypia was not conspicuous but mitotic figures and coagulative necrosis were easily identified. On the other hand, PA were composed of tumor cells with clear to eosinophilic cytoplasm, forming glands, trabeculae or nests. Most of them (35/40) had intact fibrous capsule. Mitotic figures were rarely encountered and tumor necrosis was absent. Immunohistochemical study showed that the tumor cells in PTC were positive for CK19 (11/11), chromogranin A (9/11), synaptophysin (7/11) and parathyroid hormone (11/11). They were negative for thyroglobulin, TTF-1 and calcitonin. The Ki-67 index was less than 10% (range = 2% to 9%). In contrast, the tumor cells in PA were positive (40/40) for CK19, chromogranin A, synaptophysin and parathyroid hormone. They were negative for thyroglobulin, TTF-1 and calcitonin. The Ki-67 index was less than 3%. Follow up-data were available in 9 cases of PTC (duration of follow up = 11 months to 224 months) and 7 of the patients were still alive. Follow up of all PA cases showed no evidence of recurrence.

CONCLUSIONSPTC is a rare malignant endocrine tumor presenting as neck mass. Histologic features suggestive of malignant behavior include presence of coagulative tumor necrosis and capsular/vascular invasion. It needs to be distinguished from other entities such as parathyroid adenoma, papillary thyroid carcinoma and medullary thyroid carcinoma.

Adenoma ; metabolism ; pathology ; Adult ; Carcinoma ; metabolism ; pathology ; Carcinoma, Neuroendocrine ; Carcinoma, Papillary ; Chromogranin A ; metabolism ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Humans ; Hypercalcemia ; etiology ; Hyperparathyroidism ; etiology ; Immunohistochemistry ; Keratin-19 ; metabolism ; Male ; Middle Aged ; Osteoporosis ; etiology ; Parathyroid Hormone ; metabolism ; Parathyroid Neoplasms ; complications ; metabolism ; pathology ; surgery ; Synaptophysin ; metabolism ; Thyroid Neoplasms ; metabolism ; pathology

No abstract available.
Acute Kidney Injury/diagnosis/*etiology/therapy ; Biopsy ; Fluid Therapy ; Glucocorticoids ; Humans ; Hypercalcemia/diagnosis/*etiology/therapy ; Male ; Middle Aged ; Multimodal Imaging ; Positron-Emission Tomography ; Renal Dialysis ; Sarcoidosis/*complications/diagnosis/therapy ; Subcutaneous Tissue/pathology ; Tomography, X-Ray Computed ; Treatment Outcome

Primary hyperparathyroidism due to ectopic parathyroid adenomas can pose diagnostic and management challenges, especially when imaging studies have localised the lesions to different sites. We report a case of symptomatic hypercalcaemia due to a mediastinal parathyroid adenoma. Ultrasonography identified a nodule posterior to the right thyroid gland. However, computed tomography and technetium-99m sestamibi scintigraphy revealed an ectopic parathyroid adenoma located in the anterior mediastinum. The adenoma was successfully removed through a median sternotomy. However, postoperatively, the patient developed prolonged symptomatic hypocalcaemia, possibly due to suppression of the normal parathyroid gland function, although the presence of concomitant hungry bone syndrome was possible. The histopathology of the mediastinal mass was consistent with a parathyroid adenoma.
Calcium ; blood ; Female ; Humans ; Hypercalcemia ; etiology ; Hyperparathyroidism ; diagnosis ; Hypocalcemia ; drug therapy ; etiology ; Mediastinal Neoplasms ; diagnosis ; diagnostic imaging ; surgery ; Middle Aged ; Parathyroid Glands ; pathology ; Parathyroid Neoplasms ; diagnosis ; diagnostic imaging ; surgery ; Postoperative Complications ; Technetium Tc 99m Sestamibi ; pharmacology ; Tomography, X-Ray Computed ; Ultrasonography

OBJECTIVETo investigate the diagnosis and surgical treatment of parathyroid carcinoma.

METHODSThe clinical data of 9 cases of parathyroid carcinoma treated from January 1967 to December 2009 was analyzed retrospectively with the review of related Chinese literatures.

RESULTSParathyroid carcinoma accounted for 8.9% (8/90) of all patients with primary hyperparathyroidism in our hospital, and the other one case was transferred from another hospital. Of the patients, 8 cases were found with primary hyperparathyroidism. Primary surgery was carried out with small incision: 5 patients underwent en bloc resection, among which, 3 cases received central lymph node dissection; 2 patients received simple parathyroidectomy; one case underwent palliative tumor resection. The case from another hospital received subtotal thyroidectomy. Considering preoperative, intraoperative data and frozen sections pathology, all patients were diagnosed as parathyroid carcinoma. Nine patients were followed-up for 1 - 14 years, no recurrence occurred, and the patient received palliative resection died from carcinoma two years after the operation. In previous Chinese literatures and this group, there were total 146 patients reported as parathyroid carcinoma. Those patients were diagnosed through routine histopathology, accounted for 1.8% - 11.5% of patients with primary hyperparathyroidism.

CONCLUSIONSThe diagnosis of parathyroid carcinoma is established according to severe hypercalcemia, clinical features, subset B-ultrasound and Tc(99m)-sestamibi scanning, intraoperative finding of adherence to close structures and histopathology. The initial surgical procedure of choice is en bloc resection of the tumor by minimally invasive small incision, including adjacent structures and ipsilateral thyroidectomy. The prognosis is favorable after the operation.

Adult ; Aged ; Female ; Follow-Up Studies ; Humans ; Hypercalcemia ; diagnosis ; etiology ; Hyperparathyroidism, Primary ; diagnosis ; etiology ; Lymph Node Excision ; Male ; Middle Aged ; Parathyroid Neoplasms ; complications ; diagnosis ; pathology ; therapy ; Parathyroidectomy ; methods ; Retrospective Studies ; Technetium Tc 99m Sestamibi

Biopsy, Fine-Needle ; Female ; Humans ; Hypercalcemia ; etiology ; Mediastinal Cyst ; diagnosis ; diagnostic imaging ; pathology ; Middle Aged ; Parathyroid Diseases ; diagnosis ; diagnostic imaging ; pathology ; Ultrasonography

OBJECTIVEIbandronate, a third generation bisphosphonate, inhibits bone resorption in human and animal studies. This study is to evaluate the efficacy and safety of ibandronate as a single agent in patients with tumor-associated hypercalcemia.

METHODSAn open, multicenter, non-controlled clinical trial was conducted in 22 patients. The patients received 2 mg ibandronate intravenously if the corrected calcium was less than 3.0 mmol/L but more than 2.7 mmol/L; they received 4 mg ibandronate iv if corrected calcium was more than 3.0 mmol/L.

RESULTSThere was 100% efficacy in these two dose groups but the calcium correcting effect was more pronounced in the 4-mg dose group than the 2-mg dose group. The most common adverse reactions were fever and skin itching with an incidence of 4.5%.

CONCLUSIONIbandronate is active in patients with tumor-associated hypercalcemia and the adverse effects are well tolerated.

Bone Neoplasms ; complications ; secretion ; Breast Neoplasms ; complications ; pathology ; Calcium ; blood ; Diphosphonates ; administration & dosage ; therapeutic use ; Dose-Response Relationship, Drug ; Drug Administration Schedule ; Female ; Fever ; chemically induced ; Humans ; Hypercalcemia ; blood ; drug therapy ; etiology ; Lung Neoplasms ; complications ; pathology ; Male ; Middle Aged ; Multiple Myeloma ; complications ; Phosphorus ; blood ; Pruritus ; chemically induced

Most of primary hyperparathyroidism results from parathyroid adenoma, and is characterized by hypercalcemia, reduced bone density, frequent renal stone, gastric ulcer, duodenal ulcer, muscle weakness, depression, hypertension, anemia, and rarely brown tumor. We had a case of an 80-year-old man having brown tumor caused by primary hyperparathyroidism on the right 10th rib confirmed by CT guided bone biopsy. The patient presented with decreased level of consciousness, acute gastric ulcer, acute duodenal ulcer, GB stones, renal insufficiency, depression, and osteoporosis. Serum calcium level was 16.7 mg/dL and the intact parathyroid hormone level was 3901pg/mL. A parathyroid mass was detected by neck CT and 99mTc-tetrofosmin parathyroid scan. The parathyroid tumor was removed and confirmed as a parathyroid adenoma by pathology. After operation, the patient was treated with vitamin D and calcium in response to the resulting hungry bone syndrome. The intact PTH level returned to a normal range after the removal of the parathyroid adenoma.
Aged, 80 and over ; Anemia ; Biopsy ; Bone Density ; Calcium ; Consciousness ; Depression ; Duodenal Ulcer ; Humans ; Hypercalcemia* ; Hyperparathyroidism, Primary ; Hypertension ; Muscle Weakness ; Neck ; Osteoporosis ; Parathyroid Hormone ; Parathyroid Neoplasms* ; Pathology ; Reference Values ; Renal Insufficiency ; Ribs ; Stomach Ulcer ; Vitamin D