Parathyroid carcinoma (PC) is one of the rarest malignancies making approximately 0.005% of all cancers. It may arise sporadically or less commonly, in conjunction with genetic endocrine syndromes. Due to the rarity of the disease, no general consensus or definitive guidelines exist for its pre-operative diagnosis, management, or follow up. Surgical tumor removal is the gold standard treatment to prevent its recurrence. Parathyroid carcinoma has a high recurrence rate ranging from 40 to 60% in recent literature. We report a case of a seventy-year-old elderly female with locally advanced parathyroid carcinoma successfully surgically excised completely with a 3 year disease free survival period without adjuvant chemotherapy or radiotherapy

Abbas, Syed Akbar

Ayub, Bushra

Diwan, Asad

Qureshi, Talha Ahmed

Sheikh, Shafqat Ali

Sohail, Abdul Ahad

Tariq, Muhammad Usman

English

eCommons@AKU

eCommons@AKU Department of Surgery Department of Surgery 7-2020 A rare case of three years disease free survival in a locally advanced parathyroid carcinoma successfully excised by complete surgical resection Abdul Ahad Sohail Aga Khan University, abdul.ahad@aku.edu Bushra Ayub Patel Hospital, Pakistan Syed Akbar Abbas Aga Khan University, akbar.abbas@aku.edu Shafqat Ali Sheikh Patel Hospital, Pakistan Talha Ahmed Qureshi Patel Hospital, Pakistan See next page for additional authors Follow this and additional works at: https://ecommons.aku.edu/pakistan_fhs_mc_surg_surg  Part of the Oncology Commons, Pathology Commons, and the Surgery Commons Recommended Citation Sohail, A. A., Ayub, B., Abbas, S. A., Sheikh, S. A., Qureshi, T. A., Tariq, M., Diwan, A. (2020). A rare case of three years disease free survival in a locally advanced parathyroid carcinoma successfully excised by complete surgical resection. Annals of Medicine and Surgery, 57, 62-65. Available at: https://ecommons.aku.edu/pakistan_fhs_mc_surg_surg/852 Authors Abdul Ahad Sohail, Bushra Ayub, Syed Akbar Abbas, Shafqat Ali Sheikh, Talha Ahmed Qureshi, Muhammad Usman Tariq, and Asad Diwan This report is available at eCommons@AKU: https://ecommons.aku.edu/pakistan_fhs_mc_surg_surg/852 Contents lists available at ScienceDirectAnnals of Medicine and Surgeryjournal homepage: www.elsevier.com/locate/amsuCase ReportA rare case of three years disease free survival in a locally advancedparathyroid carcinoma successfully excised by complete surgical resectionAbdul Ahad Sohaila,∗, Bushra Ayubb, Syed Akbar Abbasc, Shafqat Ali Sheikhb,Talha Ahmed Qureshib, Muhammad Usmand, Asad Diwanda Department of Surgery, Aga Khan University Hospital, Pakistanb Patel Hospital, Pakistanc Aga Khan University Hospital, PakistandDepartment of Histopathology, Aga Khan University Hospital, PakistanA R T I C L E I N F OKeywords:Parathyroid carcinomasPrimary hyperparathyroidismComplete surgical resectionThyroid lobectomyA B S T R A C TParathyroid carcinoma (PC) is one of the rarest malignancies making approximately 0.005% of all cancers. Itmay arise sporadically or less commonly, in conjunction with genetic endocrine syndromes. Due to the rarity ofthe disease, no general consensus or definitive guidelines exist for its pre-operative diagnosis, management, orfollow up. Surgical tumor removal is the gold standard treatment to prevent its recurrence. Parathyroid carci-noma has a high recurrence rate ranging from 40 to 60% in recent literature. We report a case of a seventy-year-old elderly female with locally advanced parathyroid carcinoma successfully surgically excised completely witha 3 year disease free survival period without adjuvant chemotherapy or radiotherapy.1. IntroductionParathyroid carcinoma (PC) is one of the rarest malignanciesmaking approximately 0.005% of all cancers [1,2]. Recent literaturesuggests that it accounts for only 0.5–5% of all the cases of primaryhyperparathyroidism, with less than 1000 cases reported in the litera-ture since it was first discovered in 1904 [3–5]. It may arise spor-adically or less commonly, in conjunction with genetic endocrine syn-dromes such as multiple endocrine neoplasia (MEN) type 1, type 2A,and hyperparathyroidism jaw-tumor syndrome (HPT-JT) [6–8]. Diag-nosis is usually difficult and challenging due to the absence of clinicaland radiological characteristics that reliably distinguish benign frommalignant disease [1,2,6]. Due to the rarity of the disease, no generalconsensus or definitive guidelines exist for its diagnosis, management,or follow up [6,8,9].According to recent literature, 90% of parathyroid carcinoma casesare hormonally functional on presentation with clinically apparentsymptoms of hypercalcemia and severe primary hyperparathyroidismwhich include anxiety, depression, loss of appetite, and weight lossusually present with the renal and skeletal disease [4,6,10]. Serumcalcium is often elevated with greater than 14mg/dl and serum para-thyroid hormone is often raised to about three to ten times the upperlimit of normal [1]. Surgery is the mainstay of treatment with completeresection of the tumor to achieve microscopically negative margins toprevent recurrence [1,2,6,7,11].We report a case of an elderly female who presented to us withinitial suspicion of parathyroid adenoma. However, post-operativelyshe was diagnosed with parathyroid carcinoma on histopathologicalexamination.2. Case reportThis is a case of 70 years old female, with no known co-morbidities,who presented to the Otolaryngology clinic with complaints of in-creased somnolence and reduced appetite for past the 3–4 years. Thesecomplaints were not associated with any other associated symptoms.Her past medical history and family history were unremarkable. Shehad a history of the cesarean section under spinal anesthesia 30 yearsago. On examination, she was well oriented to time, place, and person.She had a heart rate of 64 beats per min, a respiratory rate of 16 breathsper min, a blood pressure of 110/70 mm Hg, and she was afebrile. Hergeneral physical and systemic examination was also unremarkable. Herserum calcium was 14.1mg/dl (8.8–10.6) and serum parathyroid hor-mone showed a value of 457mg/dl (7-53). Her vitamin D level, serumalbumin, and creatinine were all within a normal range. 24-hour ur-inary calcium and phosphorus were also within normal limits. Shehttps://doi.org/10.1016/j.amsu.2020.07.023Received 9 June 2020; Received in revised form 12 July 2020; Accepted 13 July 2020∗ Corresponding author. Aga Khan University Hospital, Pakistan.E-mail addresses: abdul.ahad@aku.edu (A.A. Sohail), bushraayub05@gmail.com (B. Ayub), akbarabbas2000@yahoo.com (S.A. Abbas),alishafqat17@yahoo.com (S.A. Sheikh), talhaqureshi@yahoo.com (T.A. Qureshi), Muhammad.usman@aku.edu (M. Usman), asad.diwan@aku.edu (A. Diwan).Annals of Medicine and Surgery 57 (2020) 62–652049-0801/ © 2020 The Author(s). Published by Elsevier Ltd on behalf of IJS Publishing Group Ltd. This is an open access article under the CC BY license (http://creativecommons.org/licenses/BY/4.0/).Tunderwent neck ultrasonography which showed a complex solid and acystic nodule on the left side close to the lower pole of the thyroidmeasuring 3.6 × 2.8 cm (Fig. 1).The clinical picture was suggestive of a parathyroid adenoma andshe was admitted electively for its resection. After relevant investiga-tions and anesthesia fitness, she underwent complete excision of the leftparathyroid lesion and left lobectomy of thyroid gland under generalanesthesia. Intra-operative findings were enlarged left parathyroidgland which was inseparable from the multi-nodular left lobe of thethyroid gland. A recurrent laryngeal nerve was identified and saved.Neck drain was placed and the sample was sent for histopathology(Fig. 2). The lesion was not involving any other structure other than thethyroid gland. No level VI lymphadenopathy was observed in-traoperatively.The patient remained hemodynamically stable with no signs of re-current laryngeal nerve palsy. Her post-op serial serum calcium mon-itoring was done which decreased to normal range and remainednormal. No other signs of hypocalcemia were noted and serum calciumwas within a normal range. The patient remained stable and hence wasdischarged as post-op day 2 on oral calcium supplements and analgesia.At 1-week follow-up her serum calcium was 8.9mg/dl and serumparathyroid hormone was 16.3pg/ml. Histopathology revealed a neo-plastic lesion composed of closely space clusters of medium-sized ovoidto polygonal cells with pale to mildly eosinophilic cytoplasm, distinctcell borders, and centrally placed round to ovoid nuclei showing pro-minent nucleoli. Moderate cytological atypia and nuclear pleo-morphism were seen. Occasional mitotic figures were consistent withparathyroid carcinoma. The tumor was seen invading into the thyroidparenchyma 0.4cm away from the resection/capsular margin(Fig. 3).Her repeat serum calcium was also within the normal range.MEN syndrome workup was done which was negative. The patient wasseen regularly for 1 year and then has been on regular follow up yearlyfor past three years. She has been doing well with no symptoms andsigns for recurrence of disease. Hence she has an overall disease freesurvival of three years with only complete surgical resection of tumorand no chemotherapy or radiotherapy.3. DiscussionParathyroid carcinoma (PC) is one of the rarest endocrine malig-nancies with an incidence of 0.015 per 100,000 population in theUnited States [1,12]. These tumors are hormonally functional with lessthan 38 cases reported in the literature for non-functional parathyroidcarcinoma [6]. Hence patients often present with signs and symptomsof hypercalcemia hyperparathyroidism [6]. Multiple case series suggestthe involvement of renal and skeletal disease in up to 50% of patients inparathyroid carcinoma with an incidence of nephrolithiasis and renalinsufficiency as high as 56 and 84% respectively [13]. Recent studiesalso suggest the presence of a palpable cervical mass in about 30–76%of patients while the incidence of completely asymptomatic parathyroidcarcinoma described in 7–46% of patients and approximately 25% ofpatients develop distant metastases at some point during the disease[6,18]. In contrast, our patient had a hormonally functioning para-thyroid carcinoma but she did not develop renal or skeletal diseasedespite advanced age and did not have a palpable neck mass.The pre-operative diagnosis of parathyroid carcinoma is difficultand challenging due to the absence of diagnostic criteria and a lack ofconsensus in literature [8,9]. It should be suspected in a patient with avery high serum calcium level of greater than 14mg/dl or an increasedparathyroid hormone of greater than three to ten times of upper limit ofnormal palpable cervical lymph nodes should also raise suspicion of PC[9,14]. Neck ultrasonography and parathyroid scintigraphy have beendescribed as the imaging of choice for detecting parathyroid lesions anda combination of both raises diagnostic accuracy and sensitivity; how-ever, they cannot reliably distinguish benign from malignant disease[6,8,11]. A size of greater than 3cm on ultrasonography should raise asuspicion of PC [15]. Fine needle aspiration cytology should be avoidedin lesions that are suspected to be PC as it increases the risk of needletract dissemination, parathyromatosis, and recurrence [6]. Hence thepost-operative diagnosis of parathyroid carcinoma on histopathologicalexamination appears to be a norm rather than the exception [6].The gold standard for the treatment of parathyroid carcinoma iscomplete surgical excision of the tumor achieving microscopically ne-gative margins. There is a limited role of chemotherapeutic agents andradiotherapy in uncomplicated cases [6,7,9,16]. There is a lack ofevidence on prophylactic neck dissection. The incidence of neck me-tastasis has been reported to be 1–6% [6].There is a high rate of locoregional recurrence rate ranging from 40to 60% with nearly half of patients developing distant metastasis[7,17]. Recurrence of the tumor is common within 2–3 years of initialsurgery. A close follow up and tumor surveillance is required. Serialneck ultrasonography, serum calcium, phosphate, and parathyroid le-vels are required [6] 5- year disease-free survival rate after completeFig. 1. A 3.6 × 2.8cm complex solid and cystic nodule is seen on left side closeto left lower margin of thyroid measuring.Fig. 2. Parathyroid carcinoma along with thyroid lobe after resection.A.A. Sohail, et al. Annals of Medicine and Surgery 57 (2020) 62–6563surgical removal ranges from 76% to 85% [6]. This data is similar toour case described here as our patient has a disease free survival ofthree years after only complete surgical excision of tumor without ad-juvant chemotherapy or radiotherapy and is still on regular follow ups.4. ConclusionThe case of parathyroid carcinoma described here presents twounique features. Parathyroid cancer may present as a parathyroidadenoma and hence complete surgical excision of suspicious malignantlesion intra-operatively and close follow up post-operatively can pro-vide an excellent long term disease free survival rate in patient withoutadjuvant chemotherapy or radiotherapy. Secondly, complications as-sociated with hyperparathyroidism may be absent in parathyroid car-cinoma. Therefore clinicians should have a high index of suspicion forparathyroid malignant lesions if the imaging shows unusual char-acteristics. Thyroid lobectomy should be performed to enhance theclearance of malignant disease whenever deemed necessary.Funding sourcesNone.ConsentWritten informed consent was obtained from the patient for pub-lication of this case report and accompanying images. A copy of thewritten consent is available for review by the Editor-in-Chief of thisjournal on request.DeclarationThe above case has been reported in line with SCARE 2018 criteria[19].Provenance and peer reviewNot commissioned, externally peer reviewed.Ethical approvalThis case report is exempted from ethical review of our institute.Author contributionAbdul Ahad Sohail. Wrote the initial draft of case report. Collectedall relevant data from files and patient. Finalized the case report.Literature Search. Bushra Ayub: Wrote the initial draft of case report.Collected all relevant data from files and patient. Finalized the casereport. Literature Search. Syed Akbar Abbas: Was involved in criticalreview of the case report, further editing and finalizing of the case re-port. Shafqat Ali Sheikh: Was involved in critical review of the casereport, further editing and finalizing of the case report. Talha AhmedQureshi: Was involved in critical review of the case report, furtherediting and finalizing of the case report. Muhammad Usman: Was in-volved in arranging figures in case report and providing histopathologypictures. Also finalized the case report. Asad Diwan: Was involved inarranging figures in case report and providing histopathology pictures.Also finalized the case report.Registration of research studies1. Name of the registry:2. Unique identifying number or registration ID:3. Hyperlink to your specific registration (must be publicly acces-sible and will be checked):GuarantorAbdul Ahad Sohail.Syed Akber Abbas.Declaration of competing interestNone.Fig. 3. [A] Low power view of the neoplastic lesionexhibiting fibrous capsule (longer arrow), sheets ofcells and cystic spaces (shorter arrow) [B] Theneoplastic cells have pale to eosinopilic cytoplasmand vesicular nuclei with inconspicuous nucleoli[C] Few cells showed marked nuclear pleo-morphism (arrows) [D] Focal invasion of para-thyroid capsule (longer arrow) and extension intothyroid tissue is also seen (shorter arrow).A.A. Sohail, et al. Annals of Medicine and Surgery 57 (2020) 62–6564Appendix A. Supplementary dataSupplementary data to this article can be found online at https://doi.org/10.1016/j.amsu.2020.07.023.References[1] J. Goswamy, M. Lei, R. Simo, Parathyroid carcinoma, Curr. Opin. Otolaryngol.Head Neck Surg. 24 (2) (2016 Apr 1) 155–162.[2] N. Kolsi, S. Jellali, J. Koubaa, Parathyroid carcinoma: about a case and review of theliterature, The Pan African medical journal 27 (2017) 85.[3] Fd Quervain, Parastruma maligna aberrata, Deutsche Zeitschrift Fuer Chirurgie 100(1904) 334–352.[4] B. Givi, J.P. Shah, Parathyroid carcinoma, Clinical oncology 22 (6) (2010 Aug 1)498–507.[5] J.M. Sharretts, E. Kebebew, W.F. Simonds, Parathyroid cancer, InSeminars in on-cology 37 (6) (2010 Dec 1) 580–590 (WB Saunders).[6] V. Ferraro, L.I. Sgaramella, G. Di Meo, F.P. Prete, F. Logoluso, F. Minerva,M. Noviello, G. Renzulli, A. Gurrado, M. Testini, Current concepts in parathyroidcarcinoma: a single Centre experience, BMC Endocr. Disord. 19 (1) (2019 May) 46.[7] S. Storvall, E. Ryhänen, F.V. Bensch, I. Heiskanen, S. Kytölä, T. Ebeling, S. Mäkelä,C. Schalin‐Jäntti, Recurrent metastasized parathyroid carcinoma—long‐term re-mission after combined treatments with surgery, radiotherapy, cinacalcet, zole-dronic acid, and temozolomide, JBMR plus 3 (4) (2019 Apr) e10114.[8] A. Campennì, S. Giovinazzo, S.A. Pignata, F. Di Mauro, D. Santoro, L. Curtò,F. Trimarchi, R.M. Ruggeri, S. Baldari, Association of parathyroid carcinoma andthyroid disorders: a clinical review, Endocrine 56 (1) (2017 Apr 1) 19–26.[9] S. Pramanik, S. Ray, R. Bhattacharjee, S. Chowdhury, Parathyroid carcinoma andpersistent hypercalcemia: a case report and review of therapeutic options, Saudijournal of medicine & medical sciences 6 (2) (2018 May) 115.[10] W.C. Dudney, D. Bodenner, B.C. Stack Jr., Parathyroid carcinoma, Otolaryngol ClinN Am 43 (2) (2010) 441–453 xi.[11] N.T. Thanseer, A.S. Parihar, A. Sood, S.K. Bhadada, D. Dahiya, P. Singh, B.R. Mittal,Evaluation of recurrent parathyroid carcinoma: a new imaging tool in uncommonentity, World J. Nucl. Med. 18 (2) (2019 Apr) 198.[12] S.A. Hundahl, et al., Two hundred eighty-six cases of parathyroid carcinoma treatedin the U.S. Between 1985-1995: a national cancer data base report. The Americancollege of surgeons commission on cancer and the American cancer society, Cancer86 (3) (1999) 538–544.[13] A.G. Wynne, et al., Parathyroid carcinoma: clinical and pathologic features in 43patients, Medicine (Baltim.) 71 (4) (1992) 197–205.[14] T. Obara, Y. Fujimoto, Diagnosis and treatment of patients with parathyroid car-cinoma: an update and review, World J. Surg. 15 (1991) 738–744.[15] J.H. Kwon, et al., Neck ultrasonography as preoperative localization of primaryhyperparathyroidism with an additional role of detecting thyroid malignancy, Eur.J. Radiol. 82 (1) (2013) e17–21.[16] E. Shane, Parathyroid carcinoma, Curr Ther Endocrinol Metab 5 (1994) 522–525.[17] K. Sandelin, O. Tullgren, L.O. Farnebo, Clinical course of metastatic parathyroidcancer, World J. Surg. 18 (4) (1994) 594–598 discussion 599.[18] J.M. Sharretts, E. Kebebew, W.F. Simonds, Parathyroid cancer, Semin. Oncol. 37 (6)(2010) 580‐590.[19] R.A. Agha, M.R. Borrelli, R. Farwana, K. Koshy, A. Fowler, D.P. 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A rare case of three years disease free survival in a locally advanced parathyroid carcinoma successfully excised by complete surgical resection

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A rare case of three years disease free survival in a locally advanced parathyroid carcinoma successfully excised by complete surgical resection

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