Intracranial metastatic parathyroid carcinoma: case report

BACKGROUND: Although parathyroid carcinoma is not frequent, it is a slowly progressive disease characterized by frequent recurrences. A review of the literature revealed only 2 other cases of intracranial metastatic parathyroid carcinoma. We present here the case of cerebral metastases from parathyroid carcinoma that could be treated successfully. CASE DESCRIPTION: This 61-year-old Japanese woman presented to our institute with a complaint of right lower-extremity weakness after hemiconvulsion. She had undergone a parathyroidectomy for parathyroid carcinoma 18 years earlier. Lung metastasis was also detected 6 years earlier, and she has been dialyzed twice a month after chemotherapy. Computed tomographic scans demonstrated 2 enhancing right frontal tumors. After resection of the intracranial metastases, her right hemiparesis and secondary hyperparathyroidism resolved. CONCLUSIONS: This case report supports aggressive surgical management to eliminate all parathyroid hormone-secreting malignant tissue and prevent metabolic complications.     

Successful palliation of hypercalcemia secondary to metastatic parathyroid cancer: an unusual indication for hepatic resection

Primary carcinoma of the parathyroid gland is a rare disease. It is often diagnosed after recurrence of hyperparathyroidism following resection for presumed adenomatous disease. Local and distant recurrence is high and aggressive resection is advocated. Patients with parathyroid cancer are frequently plagued by severe hypercalcemia, which is often refractory to medical therapy. Herein we describe the case of a patient with metastatic parathyroid cancer localized to the liver. The patient was treated with a palliative hepatic resection for the management of persistent and refractory hypercalcemia. Intraoperative parathyroid hormone levels were utilized as an adjunct to determine successful metastatectomy. Our case highlights the importance of an aggressive approach to patients with metastatic parathyroid cancer, as well as the utility of intraoperative parathyroid hormone levels to confirm successful extirpation of disease. the authors declare no conflicts of interest     

Resection of pulmonary metastasis from parathyroid carcinoma

The management of metastasis from parathyroid carcinoma (PC) is an unusual challenge. Systemic therapy has minimal effect on the course of the disease and its associated hypercalcemia. Resection of isolated pulmonary metastases is an attractive option in the setting of recurrent hypercalcemia. We report a case of a patient who underwent resection of multiple functional PC pulmonary metastases almost 20 years after the original neck resection. The patient originally presented in 1982 with what was thought to be hyperparathyroidism secondary to a parathyroid adenoma. Surgical exploration revealed the presence of a well differentiated PC. The patient was transiently normocalcemic, but underwent surgical exploration in 1993 and 1996 for recurrent PC in the neck. The patient again improved, but presented in 2001 with diffuse arthralgia, fatigue, malaise, weight loss, and decreased appetite. She was found to have a serum calcium of 12.7 mg/dL and an intact parathyroid hormone of 1542 pmol/L. Computed tomography identified the presence of two right-sided and two left-sided pulmonary masses. Sternotomy with bilateral pulmonary metastectomy was performed. Functional PC metastasis was confirmed on pathological examination. One year following metastectomy the patient is hypocalcemic, requiring oral calcium and vitamin D supplementation. Functional pulmonary metastasis from PC is a rare cause of hypercalcemia. Complete resection of isolated pulmonary metastases can provide effective palliation with long-term survival.     

Parathyroid carcinoma. A report of five cases

Parathyroid carcinoma, although rare, represents 0.5-4.0 per cent of the cases of hyperparathyroidism. The authors have encountered five cases of parathyroid carcinoma in their experience treating 445 patients with primary hyperparathyroidism (1.1%) since 1965. All five patients were women, ranging in age fro 29 to 60 years, and had marked hypercalcemia at the time of presentation, with values up to 26 mg/dl in one case. All the tumors were locally adherent to surrounding structures at the time of parathyroidectomy. Pathologically they were characteristic with a dense sclerotic reaction and fibrous bands criss-crossing islands of parathyroid cells. Three patients with local as well as distant metastases have died as a result of recurrent parathyroid carcinoma (follow-up: 2, 3, and 6 years). The remaining two patients became normocalcemic following resection, and have been asymptomatic for 1 and 7 years, respectively. The overall survival and clinical course, in our limited experience with five parathyroid carcinoma cases demonstrates that the morbidity and mortality in these patients is mainly due to local recurrence with the resultant metabolic disturbances, as has been previously noted in the literature.     

Lung metastasis from parathyroid carcinoma causing recurrent renal hyperparathyroidism in a hemodialysis patient: Report of a case

We report herein the case of a 46-year-old woman on hemodialysis (HD) who developed recurrent renal hyperparathyroidism induced by lung metastasis from parathyroid carcinoma. The patient had been commenced on HD for chronic renal failure about 20 years earlier and had undergone a parathyroidectomy for advanced renal hyperparathyroidism 8 years later. After the initial operation, further explorations of the neck were performed due to recurrence, despite which the hyperparathyroidism persisted and she was finally referred to our department. The appearance of multiple coinlike lesions ona chest X-ray and computed tomography led to the diagnosis of recurrent hyperparathyroidism induced by lung metastasis from parathyroid carcinoma. A pulmonary wedge resection was performed and the metastatic parathyroid nodules were removed. Of the several hypotheses about the etiology of parathyroid carcinoma in HD patients, it is most likely that the parathyroid hyperplasia induced by chronic renal failure develops into carcinoma. Even in renal hyperparathyroidism, we should bear in mind the possibility that metastatic parathyroid carcinoma is a possible source of excess parathyroid hormone secretion at recurrence.     

Metachronous spread of parathyroid carcinoma to a retropharyngeal lymph node

BACKGROUND: Parathyroid carcinoma is rare and accounts for approximately 1% of patients with hyperparathyroidism. It is a slowly progressive disease with frequent recurrence and high incidence of local metastasis. Because of the rarity of this tumor, little is known about the pattern of lymph node metastasis. METHODS: A case of retropharyngeal lymph node metastasis found 2 years after initial diagnosis of parathyroid carcinoma is presented and discussed. RESULTS: A 67-year-old man initially was seen with asymptomatic hypercalcemia, and a 1.7-cm mass was removed along with the left hemithyroid. The mass was found to be parathyroid carcinoma. Serum calcium was again elevated 1 year later, and left neck lymph node dissection revealed an upper jugular lymph node containing parathyroid carcinoma. The patient continued to have an elevated serum calcium level, and a retropharyngeal lymph node was found by MRI and removed the following year. The patient is now without evidence of hyperparathyroidism or recurrent disease at 2-year follow-up. CONCLUSIONS: Persistent parathyroid carcinoma is often a clinical challenge because of the difficulty in localizing recurrent tumor. Retropharyngeal lymph nodes should be considered as a possible site of metastasis in patients with parathyroid carcinoma.     

甲状旁腺癌的诊断和治疗(附9例分析)

目的 探讨甲状旁腺癌的诊断和治疗.方法 回顾分析1967年1月至2009年12月收治的9例甲状旁腺癌的诊治经验并总结国内文献报道的部分甲状旁腺癌的相关病例资料.结果 本组9例中8例为本院病例,占本院原发性甲状旁腺功能亢进患者的8.9%(8/90),1例为外院手术后转入.8例伴重度甲状旁腺功能亢进,1例无功能;据术前资料、术中探查和冰冻病理检查诊断为甲状旁腺癌共8例;院外1例误诊.初次手术采用颈部小切口下直接手术:2例甲状旁腺肿瘤切除;5例行肿瘤扩大切除术,其中3例加中央区淋巴结清扫术;1例行甲状旁腺癌姑息性切除术;外院1例仅行甲状腺次全切除术,后追加中央区淋巴结清扫术.均经术后病理确诊为甲状旁腺癌,无颈淋巴结转移.随访1～14年,8例无癌复发,1例姑息手术者术后2年死亡.国内相关文献中报道甲状旁腺癌加本组共146例,在原发性甲状旁腺功能亢进中发生率为1.8%～11.5%.结论 根据重度高钙血症的临床表现、B超、Tc99m-sestemibi检查、术中探查见肿瘤与周围粘连和病理检查结果可诊断为甲状旁腺癌;初次手术方法宜采用包括同侧相邻组织和甲状腺在内的肿瘤扩大切除术,可用微创小切口手术施行,预后良好.     

Diagnostic dilemma of an unsuspected hyperfunctioning accessory parathyroid gland after total parathyroidectomy with autotransplantation in a peritoneal dialysis patient

Persistent or recurrent hyperparathyroidism in patients with chronic renal failure may be a frustrating problem. We report a case history of a peritoneal dialysis patient who underwent total parathyroidectomy with autotransplantation for secondary hyperparathyroidism, developed tertiary hyperparathyroidism, and in an attempt to control hypercalcemia underwent seven partial resections of the autotransplant. Subsequently, a total excision of the parathyroid autograft was performed, but the patient continued to have hyperparathyroidism and unexpectedly was found to have a hyperplastic fifth parathyroid gland identified by thallium-technetium subtraction scan. The fifth gland was removed, and a part was implanted in the right forearm; however, the autoimplant had to be completely removed because of rapidly developing hypercalcemia. Hypercalcemia was controlled, but elevated levels of parathormone persisted. Remaining parathyroid tissue could not be found.     

Metastatic parathyroid carcinoma: dilemmas in management.

The incidence of parathyroid carcinoma in patients surgically treated for primary hyperparathyroidism at the University of Michigan Hospital was 0.4% during an 18-year period. The courses of the five patients with metastatic disease are described. Histologic reevaluation and assessment of the DNA ploidy pattern were performed in each case. Localization studies preceded all reexplorations. The number of operative procedures in each patient ranged from two to 10. Two patients are living with recurrent disease and one has been disease free for 42 months. Two patients died after 2 and 12 years, respectively. Three patients had aneuploid tumors; one had a diploid tumor. One patient had both aneuploid and diploid cell populations. Dilemmas in diagnosis, localization, and medical and surgical management were encountered in patients with metastatic carcinoma. The chosen treatment should be evaluated individually in each case because of the variability in aggressiveness of this malignancy. Surgical resection proved most effective in some of these patients for both local and distant recurrences. Bisphosphonates and gallium nitrate have been reported to be effective in controlling hypercalcemia. Only the former had some effect in one of our patients.     

Acute pancreatitis associated with primary hyperparathyroidism

Coexistence of primary hyperparathyroidism and acute pancreatitis has widely been reported in literature, but a causal relationship remains controversial. A case of acute pancreatitis as a first symptom of primary hyperparathyroidism with severe hypercalcemia is reported. In this patient a reduction of serum calcium level was obtained with medical therapy and resulted in the resolution of acute pancreatitis symptoms within 10 days. At the same time a parathyroid adenoma was clinically identified and elective parathyroidectomy was performed with complete normalization of intact parathyroid hormone and serum calcium level. At three-year follow-up, no recurrence or complications of pancreatitis were documented. The presented case suggests a cause and effect relationship between acute pancreatitis and severe hypercalcemia which should be kept in mind in the differential diagnosis of non-biliary, non-alcoholic acute pancreatitis. Reduction of hypercalcemia with medical treatment can represent a good chance for elective surgical neck exploration.     

Ectopic thymic parathyroid adenoma and vitamin D deficiency rickets: a 5-year-follow-up case report and review of literature

Sixteen patients with primary hyperparathyroidism presenting as rickets have so far been reported in the English literature. However, no report of an ectopic thymic parathyroid adenoma presenting as rickets has been published. We report a 14-year-old Caucasian American, wheelchair-ridden male who presented with signs and symptoms suggestive of vitamin D deficiency rickets subsequently confirmed by laboratory and radiological findings. Following the intramuscular administration of 125,000 U ergocalciferol (vitamin D2), he developed hypercalcemia with persistently elevated parathyroid hormone (PTH) levels suggestive of primary hyperparathyroidism. Sestamibi scan demonstrated significant uptake in the superior chest, without uptake at the normal parathyroid glands location. Surgical exploration revealed normal parathyroid glands and a thymic mass, which was removed and confirmed by pathology to be a parathyroid adenoma. With subsequent oral ergocalciferol solution and calcium carbonate therapies, the patient's symptoms resolved, blood chemistries normalized, and radiological evidence of rickets significantly improved. To our knowledge, this is the first case of an ectopic thymic parathyroid adenoma in a patient presenting with rickets. Our patient demonstrates that hyperparathyroidism-induced hypercalcemia may be masked by severe vitamin D deficiency. Prolonged treatment with ergocalciferol after removal of the parathyroid adenoma was necessary to normalize iPTH and replenish vitamin D store.     

Parathyroid carcinoma associated with polycythemia vera.

A link between hyperparathyroidism and the growth of hematopoietic stem cells is suggested by this report of a parathyroid carcinoma with polycythemia vera. A 56-year-old white woman presented with splenomegaly, a palpable neck mass and hypercalcemia, recurrent six years after resection of a parathyroid tumor. She had pancytosis with a subnormal serum concentration of erythropoietin. Radiographs showed subperiosteal erosions an dosteopenia. Nephrocalcinosis was absent. Bone biopsy showed a decreased cortical width with many intracortical osteoclasts. The cancellous bone area remained normal, but the osteoid area/bone area, osteoblast perimeter and osteoclast perimeter were increased. At surgery, a parathyroid carcinoma was found in the same location operated on previously. As in two other reported cases, postoperative improvement in the hypercalcemia was associated with remission of the blood dyscrasia. A novel finding in this case is that when the hypercalcemia eventually recurred, it was again accompanied by pancytosis. With bisphosphonate therapy, the serum intact parathyroid hormone level increased in response to a decrease in the ionized calcium level, indicating that the cancer was not autonomous. This case suggests that in the presence of the ionized hypercalcemia, the parathyroid tumor may have produced or induced production of a growth factor that can stimulate pancytosis. The differential diagnosis of polycythemia and hypercalcemia should be expanded to include parathyroid tumors in addition to hepatic, adrenal, renal, and ovarian neoplasms.     

Parathyroid carcinoma: biochemical and pathologic response to DTIC

Parathyroid carcinoma is a rare cause of hyperparathyroidism. Cure results from successful en bloc resection. However, because of its rarity, the malignant nature may not be appreciated at the initial operative procedure and as a result, definitive resection may not be accomplished. However, even with extensive en bloc resections, local recurrences do occur and patients die of metabolic derangements associated with hypercalcemia. Thus in addition to operative intervention, palliative chemotherapy may be required to control the hypercalcemia. Radiotherapy has been unsuccessful. A single case of nonfunctioning parathyroid carcinoma responding to treatment with methotrexate, Adriamycin, cyclophosphamide, and CCNU has been reported. We report a case of recurrent functioning parathyroid carcinoma treated with dacarbazine (DTIC) in which biochemical and pathologic evidence of at least a partial response was seen. The patient, a 33-year-old woman, had undergone five previous neck explorations during a 26-month period for aggressive locally recurrent disease. Before DTIC therapy the intact parathyroid hormone (PTH) level was 1032 pg Eq/ml (normal 163 to 347 pg Eq/ml) and the serum calcium level was 16.8 mg/dl (normal 8.8 to 10.0 mg/dl). After a course of DTIC there was a marked improvement in her clinical status and biochemical parameters (intact PTH 545 pg Eq/ml; serum calcium 11.8 mg/dl). For 2 months her condition stabilized, with PTH levels between 700 and 760 pg Eq/ml and serum calcium levels between 10.2 and 16.0 mg/dl. With a slowly progressive rise in biochemical parameters a second course of DTIC was initiated and a marked drop in serum calcium levels (5.7 mg/dl) occurred, but PTH levels remained unchanged. A progressive course of septicemia, malnutrition, and disseminated intravascular clotting ultimately lead to her death 4 weeks later. At autopsy examination the tumor was confined to the neck. Grossly and microscopically there was extensive central as well as peripheral necrosis of the tumor, which was thought to be the result of the cytotoxic effect of DTIC. From this experience and because of the grim prognosis in patients with recurring parathyroid carcinoma, it may be that aggressive use of chemotherapy with DTIC early in the course of treatment should be considered.     

Primary hyperparathyroidism with thyroid hemiagenesis

Thyroid hemiagenesis is a very rare anomaly. We herein report a case with right thyroid lobe agenesis, which was incidentally found during the assessment of primary hyperparathyroidism. A 42-year-old male presenting with urinary lithiasis was suspected of having primary hyperparathyroidism, and had elevated levels of both serum calcium and intact parathyroid hormone. Both computed tomography and ultrasonography demonstrated the absence of right thyroid lobe and a mass of 1 cm in diameter at the left lower pole of the thyroid. The patient underwent lower left parathyroidectomy, which confirmed the right thyroid hemiagenesis, as well as the absence of both upper and lower right parathyroid glands. The resected left lower parathyroid gland was pathologically diagnosed as adenoma. The postoperative course was favourable and he was discharged on the 2nd day after surgery, without complications.     

Paraneoplastic hypercalcemia in a patient with adenosquamous cancer of the colon.

Hypercalcemia is a well-known manifestation of paraneoplastic syndromes associated with a variety of malignancies. However, colon cancer has only rarely been associated with hypercalcemia of malignancy. We present the case of a patient with recurrent adenosquamous carcinoma of the ascending colon found to have hypercalcemia. The patient is a 76-year-old white woman who initially presented with colon cancer in the cecum and underwent a right hemicolectomy. All lymph nodes and surgical margins were free of tumor. Pathological examination at that time revealed adenosquamous carcinoma of the colon. Eight months later she complained of dizziness, anorexia, and constipation and was found to have a calcium level of 13.6 mg/dL. CT scan revealed a mass measuring 10.5 to 12.7 cm in the right hepatic lobe, and a bone scan was normal. Her intact parathyroid hormone (PTH) level was 6 pg/mL (normal 12-72) and her PTH-related protein (PTHrP) level was 25.7 pmol/L (normal <1.3). She then underwent a hepatic resection. The serum PTH, calcium, and PTHrP levels normalized after resection. Hypercalcemia of malignancy in colon cancer is rare and has an association with adenosquamous histology. The hypercalcemia is attributed to PTHrP, and here we demonstrate this in the serum and tumor specimens. The effects of PTHrP are shown to be short-lived postoperatively. We find only 14 other cases in the literature of hypercalcemia related to a colonic neoplasm, and this is the only patient reported to be surviving. The diagnosis of a paraneoplastic syndrome mediated via PTHrP should be considered when hypercalcemia is encountered in the setting of metastatic colon carcinoma.     

Parathyroid lipoadenoma: The particularities of a rare entity a case report and review of the literature

Parathyroid lipoadenoma is a rare and anusual cause of primary hyperparathyroidism. The clinical presentation usually resembles other causes of primary hyperparathyroidism and the imaging is not always contributory considering its location. However, the histologic criteria are specific.We present a case that supplements and supports the rare literature data concerning the clinical and therapeutic aspects of parathyroid lipoadenoma.The case is about a 73 years old female with a right inferior parathyroid lipoadenoma that caused biological primary hyperparathyroidism. Initially followed and treated in Rheumatology department for hypercalcemia and osteoporosis, she was sent to our structure to diagnose and possibly treat the causal etiology. After non-contributory clinical examination and ultrasound imaging, the tumor was diagnosed in the cervical CT scan. The patient underwent successful surgical removal of the lipoadenoma, confirmed postoperatively on histological analysis. The follow up showed rapid normalization of the parathormon level.Even if it’s a rare condition, the diagnosis of lipoadenoma should always be considered in front of primary hyperparathyroidism with a parathyroid lesion.     

Primary juvenile hyperparathyroidism. Report of 24 cases

AIM OF THE STUDY: Primary hyperparathyroidism usually affects elderly patients. Juvenile primary hyperparathyroidism is rare, and raises diagnostic and prognostic problems. The aim of this retrospective study on 24 patients is to establish clinical, histological, and therapeutic features of juvenile primary hyperparathyroidism. PATIENTS AND METHODS: From 1986 to 2001, 673 patients were treated for primary hyperparathyroidism in our department. Twenty four patients were younger than 30 years old (3.5%). There were 14 women and 10 men. Mean age was 23 year (14-30). Clinical manifestations, pathologics findings and postoperative results were studied. RESULTS: Sixteen patients presented a sporadic form of primary hyperparathyroidism with a single adenoma. Clinical manifestations were renal symptoms in 11 cases and acute hypercalcemia syndrome in 2 cases. Seven patients had a NEM I syndrome: parathyroid lesions were 6 hyperplasia and one adenoma. A 27 years old woman presented a recurrent familial isolated hyperparathyroidism. She was operated on 10 years before and at reoperation parathyroid carcinoma was found. Nineteen patients were cured after a post operative follow up ranging from 3 to 168 months. One patient had an asymptomatic hypercalcemia recurrence. Two patients presented permanent hypoparathyroidism treated with calcitriol and calcium. CONCLUSION: Sporadic forms represent majority of cases of juvenile hyperparathyroidism. Renal manifestations are usual. Nevertheless, multiple endocrine neoplasia type 1 has to be evocated.     

Cloacogenic anal carcinoma presenting with humoral hypercalcemia: Report of a case

We present herein the case of a 60-year-old man found to have a rare type of cloacogenic anal carcinoma. The disease was advanced and aggressive with local invasion to the prostate as well as distant lymph node metastases to the neck and paraaortic region on presentation. Therefore, a palliative abdominoperineal resection was performed, 10 weeks following which the patient developed humoral hypercalcemia. Despite treatment with hydration, furosemide, steroids, and calcitonin, serum calcium continued to rise and the patient died on the 95th postoperative day. Laboratory findings revealed a decreased parathyroid hormone (PTH) level and marked elevation of parathyroid hormone-related protein (PTHRP). The detection of the PTHRP in the tumor extract and the positive immunohistochemical staining for this in the tumor cells suggested that the humoral hypercalcemia was definitely caused by its associated tumor production. Although hypercalcemia is not an uncommon complication of solid cancers in their late stage, only three other cases of rectal cancer with hypercalcemia have ever been reported. To our knowledge, this is the first documentation of cloacogenic anal carcinoma accompanied by PTHRP-induced severe humoral hypercalcemia.     

Humoral Hypercalcemia Complicating Adenocarcinoma of the Sigmoid Colon: Report of a Case

Humoral hypercalcemia can arise from a variety of malignancies, but its association with primary colorectal carcinoma is rare, with only 20 such cases documented in the English-language literature to date. We report an additional case to clarify the clinicopathologic features of colorectal carcinoma with humoral hypercalcemia. A 54-year-old woman was admitted with symptomatic hypercalcemia of 14.2 mg/dl and multiple hepatic metastases, 2 years after resection of sigmoid colon cancer. The hypercalcemia was caused by the circulating parathyroid hormone-related peptide (PTHrP) produced by poorly differentiated adenocarcinoma in the liver. The PTHrP level on admission was 13.5 pmol/l. Despite systemic chemotherapy, the patient died of disease progression 3 weeks after the humoral hypercalcemia was diagnosed. A review of the 21 reported cases, including ours, suggests that colorectal carcinoma associated with humoral hypercalcemia is characterized by a poorly differentiated tumor with or without squamous or neuroendocrine features, distant metastases, and a dismal prognosis.