Abstract. Parathyroid carcinoma is a rare disease and accounts for less than 1% of all cases of primary hyperparathyroidism. Many times, parathyroid carcinoma ...
Journal of The Association of Physicians of India ■ Vol. 65 ■ October 2017
References 1. Firestain GS, et al. Kelley’s text book of rheumatology 9th edition. Boin F. Clinical Features and Treatment of Scleroderma: Elsevier Saunders; 2013: chapter 84. Page 1366-68 2. Gajendra S, et al. Coexistence of scleroderma with multiple myeloma:a rare association. BMJ case reports Sept 2013doi:10.1136/bcr-2013-200639
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3. Stables GI, Taylor PC, Highet AS. Scleroderma associated with paraproteinaemia treated by extracorporeal photopheresis. Br J Dermatol 2000; 142:781–3 [PubMed].
6. Doyle JA, Connolly SM, Hoagland HC. Hematologic disease in scleroderma syndromes. Acta Derm Venereol 1985; 65:521–5 [PubMed]
4. Korting GW, Gilfrich HJ, Meyer zum Buschenfelde KH. Scleroderma adultorum and multiple myeloma. Arch Dermatol Forsch 1974; 248:379–85 [PubMed]
7. Kasper DL et al. Harrison’s Principles of Internal Medicine.19th edition. Munshi N C et al. Plasma cell disorder: McGraw-Hill Education; 2015.chapter 135.page 710-716.
5. Hodak E, Tamir R, David M, et al. Scleroderma adultorum associated with IgG-kappa multiple myeloma—a case report. Clin Exp Dermatol 1988; 13:271–4 [PubMed
Parathyroid Cancer Causing Acute Severe Pancreatitis Anil Pal1, Amey Sonavane1, Ritesh Agrawal2, Pravin Rathi3
Abstract Parathyroid carcinoma is a rare disease and accounts for less than 1% of all cases of primary hyperparathyroidism. Many times, parathyroid carcinoma is detected only after surgery. Parathyroid carcinoma as a cause of acute pancreatitis is uncommon. We report this case of acute severe pancreatitis associated with parathyroid carcinoma. Hypercalcemia was found during workup for acute pancreatitis which was due to primary hyperparathyroidism. During surgery, there was a suspicion of parathyroid carcinoma and en bloc resection was done followed by adjuvant radiation therapy. It is important to treat the precipitating factor for acute pancreatitis. Surgery is the mainstay of treatment for parathyroid carcinoma.
Introduction
P
arathyroid carcinomas account for less than 4% of parathyroid
diseases.1 They principally present with elevated serum calcium and PTH levels. Initial presentation very often is with a “hypercalcemic crisis”.
We r e p o r t t h i s c a s e o f a c u t e severe pancreatitis associated with parathyroid carcinoma. Hypercalcemia was found during workup for acute pancreatitis which was due to primary hyperparathyroidism. During surgery, there was a suspicion of parathyroid carcinoma and en bloc resection was done followed by adjuvant radiation therapy.
Case Report A 43-years-old gentleman, textile-mill worker, presented in e m e r g e n c y w i t h s e ve r e e p i g a s t r i c pain. Generalized weakness, reduced appetite and recurrent vomiting were present for the past 45 days. He also complained of severe, dull continuous pain in the epigastrium radiating to the back for one week which was aggravated on consuming food and was partially relieved on bending forward, associated with dyspepsia, constipation and weight loss. His urine output was reduced. On examination, he was dehydrated with persistent vomiting, afebrile and tachycardia with normotension. Abdominal examination demonstrated distension with epigastric tenderness. Blood investigations revealed leukocytosis (12,000/cu mm) with polymorphonuclear leucocytosis, elevated pancreatic enzymes- serum lipase (8605 U/L) and serum amylase (2435 U/L), hypercalcemia (14.7 mg/
Fig. 1: Tc99m Sestamibi scan with subtraction imaging for thyroid revealed a MIBI avid tumor in left superior group of gland with focal retention at 3 hours
1 Resident in Gastroenterology, 2Consultant in Endocrine and Breast Surgery, 3Consultant in Gastroenterology, Bombay Hospital and Medical Research Centre, Mumbai, Maharashtra Received: 11.06.2016; Accepted: 07.07.2017
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Journal of The Association of Physicians of India ■ Vol. 65 ■ October 2017
radiotherapy is going on.
A
Discussion Figure 3
Parathyroid carcinomas account for less than 4% of parathyroid diseases. 1 A. Neoplastic cells with They principally present with elevated intervening fibrous band (100x) serum calcium and PTH levels. Initial p r e scells e nwith t a thigh i o n ve r y o f t e n i s w i t h a B. Neoplastic N:C ratio, B “ h yhyperchromatic, percalcemic crisis”. Metastatic irregular nucleoli and clear invasion of regional lymph nodes or cytoplasm and coagulative tumor necrosis (400x) distant sites can confirm the diagnosis preoperatively. A diagnosis can also be suggested intraoperatively on the basis of tumor invasion into surrounding structures. An intraoperative diagnosis of carcinoma based on frozen section findings is rather controversial because of the overall difficulty of rendering Fig. 3: Microscopy of left superior the diagnosis. Histopathologic parathyroid gland. (A) features include a high mitotic rate Discussion Neoplastic cells with intervening and a capsular, vascular or perineural fibrous band (100x). (B) Parathyroid carcinomas account for less than 4% of parathyroid diseases.[1] They principally Neoplastic cells with high N:C invasion. 1,2 Other findings include present with elevated serum calcium and PTH levels. Initial presentation very often is with a ratio, hyperchromatic, irregular cellular pleomorphism, atypia and “hypercalcemic crisis”. Metastatic invasion of regional lymph nodes or distant sites can confirm nucleoli and clear cytoplasm atypical mitoses. The abnormal mitotic the diagnosis preoperatively. A diagnosis can also be suggested intraoperatively on the basis of and coagulative tumor necrosis figures distinctive, though not tumor invasion into surrounding structures. An intraoperative diagnosis of carcinoma basedare on (400x) frozen section findings is rather controversial because of the overall difficulty of rendering the pathognomic. Postoperative recurrence rates are high with significant 2-year superior parathyroid gland and other mortality rate between 46% and 65%. 3 3 glands were normal in size, location A s the condition is rare, leading and color. The tumor was densely authorities including the American adhered to left lobe of thyroid and en Joint Committee on Cancer (AJCC) bloc resection of left sided parathyroid have not published any staging mass (left superior parathyroidectomy recommendations. 4 with left hemithyroidectomy) and central compartment lymph node Acute pancreatitis secondary to dissection with left cervical thymectomy primary hyperparathyroidism (PHPT) was done. The gross specimen is infrequent. It was first described by (parathyroid+hemithyroidectomy) Cope et al. 5 Work-up for PHPT should weighed 18 grams. The parathyroid be routinely performed to detect the t u m o r wa s 3 . 0 x 1 . 7 x 2 . 7 c m i n s i z e etiology of non-gallstone pancreatitis (Figure 2). secondary to hypercalcemia. The prevalence of PHPT is estimated to Histopathology showed that be between 1.5% and 7%. 3 Based on parathyroid mass was a parathyroid the available epidemiological data, c a r c i n o m a a n d wa s c o m p r i s e d o f a direct causal relationship between neoplastic cells, arranged in nests and PHPT and acute pancreatitis appears separated by broad fibrous bands with to be doubtful. It is a known fact sparse mitoses and some intranuclear that hypercalcemia of any etiology inclusions with areas of coagulative can potentially, albeit rarely lead tumor necrosis. There was no perineural to acute pancreatitis. Other rare invasion or lymphovascular emboli but causes of hypercalcemia leading to adipose tissue at periphery of tumor pancreatitis include total parenteral was invaded. Tumor was also adhered nutrition, metastatic bone disease, to thyroid but not infiltrating it. All vitamin D toxicity, sarcoidosis and lymph nodes and thymus were free infusion of intravenous calcium in of disease (Figure 3). Postoperatively, high doses perioperatively during patient had normalization of serum cardiopulmonary bypass. PTH and serum Calcium denoting successful outcome of surgery. He was The suggested pathophysiological discharged on 4 th postoperative day mechanisms that cause pancreatitis in after stabilization of serum Calcium hypercalcemia include I) Deposition on oral Calcium and Vitamin D of calcium in the pancreatic duct supplements. He is asymptomatic at causing pancreatic duct obstruction; 4 months follow up and adjuvant local Microscopy of left superior parathyroid gland.
Fig. 2: The surgical specimen of parathyroid and hemithyroidectomy weighting 18 grams. The parathyroid tumor was 3.0x1.7x2.7 cm in size
dl) hypoalbuminemia (3.3 gm/dl), elevated serum parathyroid hormone (PTH) (948.7 pg/ml) and serum alkaline phosphatase level (217 U/L). Serum triglycerides were normal. Ultrasound (USG) of the abdomen revealed a bulky pancreas with peripancreatic fat stranding, normal gallbladder, intra-hepatic biliary radicles, common bile duct and n o r m a l s i z e d k i d n e y s . T h e r e wa s no free fluid in the abdomen. Based on the history, clinical examination, b l ood investig ations and USG, he was diagnosed to have acute severe pancreatitis secondary to PTHdependent hypercalcemia. He was treated aggressively with intravenous fluids, analgesics, antiemetics and proton pump inhibitors. The serum calcium levels were persistently high despite adequate hydration and diuresis, hence steroid wa s a d d e d . S e r u m c a l c i u m l e ve l s dropped but still continued to remain above the normal range. Acute pancreatitis resolved with conservative management. Localization studies were done for parathyroid, anatomical localization in form of USG neck revealed a large extrathyroidal mass at the upper pole of the left thyroid lobe measuring 2.8x2.6x1.5 cm. Functional localization by Tc 99m Sestamibi was concordant with USG and showed high uptake in same area (Figure 1). As the tumor was palpable, in an elderly male and presentation was hypercalcemic crisis, a presumptive diagnosis of parathyroid carcinoma was made. Bilateral neck exploration for parathyroid glands was done which showed tumor was arising from left
Journal of The Association of Physicians of India ■ Vol. 65 ■ October 2017
II) Activation of trypsinogen within the pancreatic parenchyma due to hypercalcemia leading to autodigestion of the pancreas; and III) Genetic variants in SPINK 1 (serine protease inhibitor Kazal type 1) and CFTR (cystic fibrosis transmembrane conductance regulator) genes along with hypercalcemia that increase the risk of acute pancreatitis in patients with PHPT. Acute pancreatitis is usually associated with a decrease in serum level of calcium and this is mainly related to decreased serum albumin levels. Based on the Ranson grading, low serum calcium levels has prognostic significance and is a marker of severity because it is carried bound to albumin-rich intravascular fluid that extravasates to the peritoneum. Hence, it is not common to observe hypercalcemia in a patient with severe acute pancreatitis. Presence of
hypercalcemia in pancreatitis should a l wa y s a l e r t t r e a t i n g p h y s i c i a n s about presence of underlying hyperparathyroidism or malignancy. 6 Parathyroid hormone levels should be tested and if hormone is elevated and imaging of the parathyroid glands should be conducted. In our case, the diagnosis of a parathyroid mass was made by USG neck followed by 99mTc-Sestamibi scintigraphy. Surgical resection of the mass is definitive treatment as parathyroidectomy protects from recurrence of pancreatitis. Postoperative hypocalcemia is common and warrants calcium supplementation.
Conclusion A multi-specialty coordinated approach between gastroenterologists, radiologists and surgeons is imperative in treating this rare phenomenon of acute
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pancreatitis caused by hypercalcemia secondary to a parathyroid carcinoma.
References 1.
Heffess CS. Embryology, anatomy, and histology. In: Wenig B. Atlas of Head and Neck Pathology. 2nd ed. 2008. China: Saunders Elsevier; 2008:1012–1028.
2. Seethala RR, Virji MA, Ogilvie JB. Pathology of the parathyroid glands. In: Leon Barnes, ed. Surgical Pathology of the Head and Neck. Vol III. New York, NY: Informa Health Care; 2009:1429–1472. 3.
Okamoto T, Iihara M, Obara T, Tsukada T. Parathyroid carcinoma: etiology, diagnosis, and treatment. World J Surgery 2009; 33:2343–2354.
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Edge SB, Byrd DR, Compton CC, Fritz AG, Greene FL, Trotti A, eds. AJCC Cancer Staging Manual. 7th ed. Chicago, IL: Springer. 2010.
5. Cope O, Culver PJ, Mixter CG Jr, Nardi GL. Pancreatitis, a diagnostic clue to hyperparathyroidism. Ann Surg 1957; 145:857- 863. 6. Braun C, Duffau P, Mahon FX, Rosier E, Leguay T, Etienne G, Michaud M. [Acute pancreatitis due to hypercalcemia revealing adult T-cell leukemia]. Rev Med Interne 2007; 28:116-119.
Lemierre’s Syndrome in Pregnancy Secondary to Retropharyngeal Abscess Joe James1, NK Thulaseedharan2, NV Jayachandran3, P Geetha3 Table 1: Investigations at admission and after 2 weeks when fever recurred
Abstract Lemierre’s syndrome (LS) refers to suppurative thrombophlebitis of internal jugular vein (IJV) secondary to oropharyngeal infection. It is caused by the anaerobic bacteria Fusobacterium necrophorum. Here we report a case of LS secondary to retropharyngeal abscess in a pregnant lady with possible underlying connective tissue disorder. A 19-year old primigravida at 6-weeks of gestation, presented with fever, cough, dyspnea, right sided neck pain and swelling. Imaging showed right lower lobe pneumonia with bilateral pulmonary infiltrates and pleural effusion. Ultrasound of the neck showed right IJV thrombosis. Magnetic resonance imaging of the neck revealed a retropharyngeal abscess extending from C1 to C4 vertebral level. She had positive ANA, SS-A and Ro-52 titres. She was treated with piperacillin-tazobactam, metronidazole, enoxaparin and short course steroids. Even though she improved initially, fever recurred and she had a massive hemoptysis with hemothorax and expired.
Introduction
L
emierre’s syndrome (LS) refers to suppurative thrombophlebitis of internal jugular vein (IJV) secondary to oropharyngeal infection. It is caused by the anaerobic bacteria Fusobacterium necrophorum. Here we report a case
of LS secondary to retropharyngeal abscess in a pregnant lady with possible underlying connective tissue disorder.
Case Report A 19-year old primigravida with 6-weeks of gestation presented with high grade fever, headache and dry
On admission Hemoglobin WBC
After 2 weeks
11.8 g/dL
9.7
10200/mm3
11,500
P76L17M7
P82L12M6
1.69×105/mm3
4.29
ESR
60 mm in 1st hr
112
CRP
61.8 mg/L
39
Blood urea
10 mg/dL
12
S. creatinine
0.7 mg/dL
0.8
Urine microscopy
Normal
Normal
S. sodium
130 meq/L
132
S. potassium
3.9 meq/L
2.9
Total bilirubin
0.7 mg/dL
0.4
Differential Platelet
Total protein
6.7 g/dL
6.4
Albumin
3.3 g/dL
3.4
A/G ratio
1.1
0.9
AST
33 IU
36
ALT
21 IU
23
Resident, 2Professor and Head of the Department, Professor, Depar tment of Internal Medicine, Government Medical College, Kozhikode, Kerala Received: 04.12.2016; Accepted: 07.07.2017 1 3
