Metastatic pheochromocytoma in the spine is rare, and there is no standard curative management. Treatment via open surgery is often risky in the perioperative period, while osteoplasty by cement augmentation is a less invasive option.We describe 2 patients with recurrence of pheochromocytoma involving the spine and the pelvis who were successfully treated with osteoplasty by cement augmentation. A 31-year-old female underwent cement augmentation for a pelvic lesion 6 months after the resection and screw fixation of an L3 lesion. A 58-year-old male underwent cement augmentation to directly destroy the functional tumor, with a surgical decompression 6 months later. Both patients showed appropriate destruction of the tumor, adequate pain relief, and the decreased release of catecholamine from metastatic lesions.Osteoplasty by cement augmentation may be a treatment option for patients with metastatic pheochromocytoma who cannot undergo appropriate surgery or decline surgery. This represents a safe approach to sustainably relieve pain and stabilize vertebral bodies with metastatic malignant pheochromocytoma. Spinal metastases are extremely rare, with various clinical presentations based on the primary lytic bone lesions that cause destruction or compression of the spinal cord, clinically manifesting as pain or neurological deficit.,2 Previous reports investigating treatment methods for malignant tumor in the spine have studied preoperative embolization of vascular supply, chemotherapy and/or radiotherapy, and/or tumor resection, and concluded that open curative resection of the sectional lesion was effective. However, open surgery carries marked challenges including hemodynamic drastic fluctuation induced by catecholamine secretion from the tumor, blood loss caused by lesion destruction, and spinal stabilization. Herein, we present 2 cases in which pheochromocytoma of the spine was successfully treated via osteoplasty by cement augmentation for the first time; this method deactivated the neuroendocrine systems, reduced perioperative blood loss, and stabilized the spine. Written informed consent was obtained from the patient for publication of this article. A copy of the written consent is available for review by the editors of MEDICINE. Because this article does not involve any human or animal trials, there is no need to conduct special ethic review and the ethical approval is not necessary.Pheochromocytomas are rare tumors with an incidence of 1 per 100,000 per year, 10% to 20% of which will develop to malignant extraadrenal pheochromocytoma.22.12.1.1A 31-year-old female with a 17-year history of hypertension (maximum blood pressure (BP) ≤200/120 mm Hg) underwent surgical resection of adrenal pheochromocytoma 5 years previously. Postoperative BP returned to normal with no evidence of distant metastasis during a 4-year follow-up. However, the patient experienced severe paroxysmal headaches provoked by hypertensive peaks, moderate low back pain and numbness of the right lower limb for 1 year before being referred to our hospital.Two extraadrenal pheochromocytomas were found, with no recurrence of the primary tumor Fig. A. The leWe initially planned a 2-step resection of the L3 and pelvic lesions. The patient was medically optimized with alpha- and beta-adrenergic blockers for 6 weeks, and showed reduced catecholamine and normal BP.2.1.2The patient was placed in the prone position under general anesthesia. We exposed the L2-L5 spinous processes and laminae, resected a 3 cm × 3 cm × 2 cm dark purple tumor mass on the right facet between L3/4 and the L3 pedicle, and performed fixation using a Moss SI screw-rod system. Perioperative BP was stable and intraoperative blood loss was 800 mL. Pathological examination was positive for chromogranin A, synaptophysin and P53, occasionally positive for S-100, but negative for melan-A and calretinin, which confirmed the diagnosis of pheochromocytoma  after cessation of antihypertensive drugs, and urine catecholamine increased to 139.50 μg/24 hour 3 months after surgery. The BP remained high (140/90 mm Hg) at the next 2-month follow-up, despite ongoing beta-blocker administration.2.1.3Six months later, the patient was too scared to undergo the previously designed resection of the pelvic lesion. The patient instead gave permission for percutaneous osteoplasty by polymethylmethacrylate to be performed under local anesthesia Fig. C. The BP2.1.4The beta-blocker medication was gradually reduced and was stopped at 2 months postoperatively. During 2 years of follow-up, both BP and urine catecholamine were normal. There were no complications associated with osteoplasty during the follow-up period.2.22.2.1A 58-year-old male underwent complete excision of right retroperitoneal pheochromocytoma with no metastasis 4 years previously. The patient experienced high BP (range 160–120/135–90 mm Hg) and progressive low back pain with radiating pain to the right lower limb for 1 year. Physical examination revealed a stiff lumbar region, right lower limb hyperesthesia, positive Lasegue's sign, and urine catecholamine of up to 151.24 μg/24 hour. Computed tomography and plain radiography revealed a soft mass outside the sacrum, as well as a compressed dural sac, which was confirmed by bone scanning  in the present study, indicating that the local malignant tumors were eradicated.Reports of spinal pheochromocytoma are rare, 35% of which are metastases. enucleated the S3 tumor posteriorly, and the patient was disease-free after 2 years of follow-up. Another case with a tumor at S1 underwent therapy with I131 metaiodobenzylguanidine continually after failure of radiotherapy, and showed no tumor growth after 5 years. A third case with an S1–S2 lesion underwent open resection and received a total dose of 3000 cGy in 10 fractions, with no follow-up. In the fourth case, the sacral tumor was effectively treated with a combination of 4 methods, including preoperative sacral embolization, percutaneous cryoablation, alcohol ablation, and sacroplasty.. In the present report, we confirmed that osteoplasty was able to relieve local pain caused by metastatic spinal pheochromocytoma in 2 cases.Only 4 sacral pheochromocytomas have been reported since 1998. Coles et alTo date, surgical management of spinal pheochromocytoma has remained under evaluation, with no standard criteria. We described the first 2 cases of metastatic spinal pheochromocytoma that underwent cement augmentation. In both cases, the local tumor was successfully controlled, with pain relief and decreased catecholamine levels. This might be a useful strategy to achieve rapid and sustained neurological improvements for patients with recurrence of pheochromocytoma involving the spine and the pelvis. Although there were no complications associated with osteoplasty in these 2 patients, the safety of this approach still needs to be confirmed in studies with larger sample sizes and longer follow-up periods. Surgical time, cement volume, and velocity are critical factors that need further investigation.


In the presentarticle, we emphasize the clinicopathological features and differentialdiagnosis of the disease.Lipomas are very common benign slow-growing soft tissue neoplasms composed ofmature adipose tissue mostly diagnosed in the fifth decade of life. These tumorsrarely present in the oral cavity, representing less than approximately 5% ofall benign mouth tumors. They are usually less than 2cm in size and etiologyremains unclear. We report a young male patient presenting with a giant lipomain the buccal mucosa. Histopathology revealed a large area of mature fat cellsconsistent with conventional lipoma and an area of the mucosal lining of thelesion suggestive of  It is most common on the trunk, shoulders, neck and axilla, beingrare on the hands, lower legs or feet. Cases involving children are veryuncommon.5 Several cases of lipomas entrapping salivary gland tissue havebeen recently described and termed sialolipoma.5 Although clinical diagnosis of OL is usually apparent,salivary gland or other mesenchymal tumors should be included in the differentialdiagnosis for the disease.4Histologically, lipomas can be classified as conventional lipoma or its variants:fibrolipoma, angiolipoma, spindle cell/pleomorphic lipoma, myxolipoma, chondroidlipoma, osteolipoma, myolipoma and intramuscular or infiltrating lipoma.We report a young male with a large lipoma in the buccal mucosa and highlight theclinicopathological features and differential diagnosis of the disease.A 29-year-old male patient was referred to the Stomatology Clinics at BauruDental School – University of São Paulo – with a chief complaintof a 1–year painless movable mass in the left buccal mucosa region. Oral examinationrevealed a nodular and pedunculated mass with defined borders, regular contour andresilient consistency . The mucClinical diagnosis was presumed to be a lipoma, and differential diagnosis includedsalivary gland tumor or other mesenchymal neoplasm. The patient signed a writtenconsent form before the surgery. We resected the lesion off the adjacent musclefibers of the buccinator under local anesthesia . The surmorsicatio buccarum. We also observed microbial biofilms onsuperficial layers  are rarely malignant, owing to the fact thatlesions grow slowly and show a normal overlying mucosa and lack of nodalinvolvement.1Lipomas rarely occur in the oral cavity. When they are present, the most common placefor their occurrence is the buccal mucosa, followed by the tongue.In this report, a large-size lesion was located in the left buccal mucosa. Althoughlipoma was our main diagnosis, we considered the possibility of different neoplasmsand salivary gland tumors as differential diagnosis of the disease. Salivary glandadenomas, for example, can occur in the palate, lips and buccal mucosa, and some ofthem – like canalicular adenoma and duct papillomas – arise almost exclusively inminor salivary glands. Pleomorphic adenoma represents up to 70% of minor glandtumors.8 In these cases, thetumors show greater recurrence rates after surgical treatment. On the other hand,although intraoral lipomas have no well-defined limits, they rarely recur. In thepresent case, we observed close contact between the lesion and the buccinator muscleduring the surgery. Transurgical observations revealed the separation of the lesionfrom the deepest muscle tissue. The microscopic sections showed a well-circumscribedlesion, separated from the muscle bundles. Therefore, we discarded the diagnosis ofintramuscular lipoma.Occasionally, lipomas may invade or grow between the muscles: the so-calledintramuscular, infiltrating lipoma.10Differential diagnosis includes lipoma, foreign body granuloma, traumatic neuromaand salivary neoplasm. This lesion generally occurs in infants andchildren.5 Oralpathologists should pay special attentions to such events before treatingpatients.Histologically, lipoma cells cannot be distinguished from a herniated buccal fat pad.The herniation of a buccal fat pad presents as an expanding pedunculated massemanating from the deep soft tissue in the buccal mucosa region, with a history ofposttraumatic sudden onset.morsicatiobuccarum. Indeed, buccal mucosa is frequently traumatized, and thepossible role of trauma in the growth of OL cannot be ruled out; nevertheless, noconsensus exists regarding the pathogenesis of OL. 10 Fatty degeneration, heredity,hormonal basis, infection, metaphase of muscle cells, lipoblastic embryonic cellnest in origin, chronic irritation and trauma are possible theories that support thepattern of a lipoma.10Microscopic analysis of the present case also supports the clinical observation ofocclusal trauma since it revealed an area consistent with 7 We couldonly find a few reported cases of this rare entity: large or giant OL.One aspect that caught our attention in the present case is that the lesion was verylarge, with 5cm at its largest diameter. Most lipomas on the body are smaller than5cm and OL are usually less than 2cm.


Background: Myeloid sarcoma (MS) or granulocytic sarcoma is a rare tumor consisting of myeloid blasts with or without maturation occurring at anatomic sites other than the bone marrow. MS can involve any organ system but shows a predilection for skin, bone, and soft tissues of head and neck region.Case report: We report four cases of MS occurring at unusual sites, out of which three were de novo and one was associated with acute myeloid leukemia (AML).Conclusion: Although MS is associated with AML, it can rarely present without any existent hematologic disease. Differential diagnosis of a soft tissue mass should include MS even in the absence of leukemia. Establishment of the correct diagnosis depends on morphologic, histochemical, and immunohistochemical examination. Myeloid sarcoma (MS), also known as extramedullary acute myeloid leukemia (AML), granulocytic sarcoma (GS) or chloroma, is a tumor composed of myeloid cells occurring at an extramedullary site , 2]. Th. Th2]. TWe report four cases of MS to highlight the unusual clinical presentation of this entity and the diagnostic dilemma it can pose Table 1 .A 38-year-old male presented with complaint of gradually progressive, left sided nasal obstruction for the last three months. Subsequently there was loss of vision in the left eye. General physical and systemic examination was unremarkable. On anterior rhinoscopy, an irregular, firm mass was seen in the left nasal cavity. Hematologic profile revealed anemia (hemoglobin = 10.4 g/dl), leucocytosis  and marked eosinophilia . Computed tomography (CT) scan showed an intranasal mass involving the bilateral ethmoid sinuses with destruction of the ethmoid septae and the medial wall of the left orbit along with an extension into the inferior extraconal compartment of the left orbit and superior displacement of the left eye ball Figure 1 . A cliniSubsequent bone marrow examination showed myeloid preponderance with marked increase in mature eosinophils and presence of eosinophilic myelocytes and metamyelocytes. However, no blasts were seen.The patient was advised chemotherapy but was lost to follow-up subsequently.An 11-year-old male child presented with proptosis of the right eye for nine months. Local examination revealed a retro orbital mass. Biopsy from the mass showed large atypical cells with vesicular nuclei, prominent nucleoli, and scant cytoplasm, admixed with myeloid precursors and lymphocytes  with an extensor plantar reflex. Routine hematologic and biochemical tests were within normal limits. Magnetic resonance imaging (MRI) of the head showed a homogenously enhancing multilobulated mass in the prepontine, premedullary and left cerebellopontine angle cistern region with mass effect over the brainstem and the left cerebral hemisphere. With a clinicoradiologic diagnosis of high grade glioma, surgery with decompression of the mass was done.Microscopic examination of the tumor tissue showed a highly cellular tumor, diffusely infiltrating the meninges and surrounding fibroadipose tissue. It consisted of mixed population of large round cells, some with indented nuclei and numerous mature and immature eosinophils , before occurrence of AML (in 0.6% patients) and in association with other myeloproliferative neoplasms like blast crisis in CML and MDS. Rarely, it occurs ur cases .There is a predilection for males and last decades of life with a median age of 56 years. MS can involve any site of the body. Skin, soft tissues, bone, lymph nodes, GIT, and testis are affected more frequently , 2]. Oc. Oc2]. OCNS and orbital involvement is commonly seen in the pediatric population. In the absence of overt leukemia, involvement of spinal cord is rare with only a few cases reported in the literature . ThoraciMS occurs in approximately 2–8% of patients with AML. Of these, 15–35% of the cases of MS can be seen concomitantly with AML, about 25% of the cases precede AML and up to 50% of the cases occur after the diagnosis of AML. It might as well be the initial manifestation of relapse in a patient who has been in remission [The most common microscopic form of myeloid sarcoma consists of a mixed population of myeloblasts, neutrophils, and neutrophil precursors. Based on the extent and degree of maturation, three major subtypes have been defined: blastic, immature, and differentiated. MS blastic type is composed primarily of myeloblasts with little evidence of maturation. The immature type is intermediate between blastic and differentiated forms and consists of myeloblasts, promyelocytes, and eosinophilic myelocytes. The differentiated or mature type is composed of promyelocytes and more mature cells with abundance of eosinophils. Myelomonocytic or pure monoblastic morphology is present in a large number of cases.MLL rearrangement, inv(16), trisomy 4, monosomy 16, 16q-, 5q-, 20q- and trisomy 11 [The diagnosis of MS is validated by the results of cytochemical and/or immunophenotypic analyses. The immunohistochemical panel includes CD68/KP1, antimyeloperoxidase (MPO), CD43, CD117, CD99, CD68/PG-M1, lysozyme, CD34,terminal deoxynucleotidyl transferase (TdT), CD56, CD30, glycophorin, and CD4. The combination of the above markers allows recognition of tumors with immature myeloid phenotype, as well as cases which show differentiation towards myelomonocytic, monoblastic, erythroid or megakaryocytic lineages. At times aberrant antigenic expression with cytokeratin (CK), B- or T-cell markers is noted. In 55% of the cases chromosomal aberrations are known to occur which can be confirmed by FISH and/or cytogenetics. These include monosomy 7, trisomy 8, In the absence of peripheral blood and bone marrow involvement MS can be misdiagnosed as a number of other neoplastic diseases depending on the site of involvement. The major differential diagnosis of MS is lymphoma including lymphoblastic lymphoma, Burkitt lymphoma, and diffuse large B-cell lymphoma. Other less common differentials are soft tissue sarcomas, small round cell tumors  and undifferentiated carcinoma. It is important to distinguish between these entities by cytochemical and immunohistochemical stains for prompt recognition and institution of appropriate treatment .The prognosis of MS depends upon the initial context in which it appears. Most patients with isolated MS progress to AML within months of diagnosis. Cases that progress to AML seldom survive more than a year .Patients with primary myeloid sarcoma should be treated as AML even in the absence of clinically detectable leukemia. The treatment modalities include induction/intensification therapy similar to those with AML. The role of radiotherapy is also described by some authors .MS is an uncommon tumor and can occur in unusual locations with variable presentation as seen in the present series. Therefore, it should be considered in the differential diagnosis of a soft tissue mass regardless of the presence/absence of leukemia. It is important to make a correct diagnosis by using the armamentarium of morphology, histochemistry, and immunohistochemistry collectively for prompt institution of therapy.The authors declare that they have no competing interests.


Intra-abdominal desmoid tumors, particularly those derived from the stomach, are rare. Such tumors are associated with a history of familial adenomatous polyposis (FAP), trauma, or surgical procedures in general. In addition, spontaneous shrinking of an intra-abdominal desmoid tumor is rarer. And desmoid tumors most commonly arise during the fourth decade of life.A 17-year-old boy with lower abdominal pain was diagnosed with a gastrointestinal stromal tumor (GIST) or a hematoma at a local hospital. He had no history of FAP, trauma, or previous surgery. Abdominal computed tomography (CT) was performed for observational purposes three times over a 9-month period. The tumor gradually decreased in size over time; however, the tumor did not shrink sufficiently to be diagnosed as a hematoma. Because there was a high possibility of a GIST from the stomach, he underwent laparotomy. Operative findings revealed that the tumor was a hard mass firmly attached to both the greater curvature of the stomach and the inferior pole of the spleen. Pathologically, the tumor was diagnosed as a desmoid tumor derived from the stomach.For a young boy without a history of FAP, trauma, or surgical procedures, it is difficult to define an intra-abdominal tumor near the stomach as a desmoid tumor. In such cases, surgical resection is recommended for a definitive diagnosis. Desmoid tumors were first described in 1832 by MacFarlane [Desmoid tumors are also reportedly associated with histories of trauma or prior surgery, particularly in patients with FAP , 5. DesmWe report a rare case of an intra-abdominal desmoid tumor derived from the stomach in a young patient without a history of FAP, trauma, or surgery.A 17-year-old boy presented to a local hospital due to lower abdominal pain in September 2014. Abdominal computed tomography (CT) revealed a solid mass measuring approximately 74 mm × 45 mm that was attached to the greater curvature of the stomach Fig. . EsophagHe was referred to our hospital for management of his GIST in December 2014. He experienced no symptoms following medical examinations conducted at the local hospital. CT performed at our hospital revealed a solid mass with slightly inhomogeneous enhancement and axis lengths of 57 mm × 44 mm, which reflected a small reduction in size compared with prior CT findings Fig. . TherefoIn March 2015, 3 months after the previous examination, a third CT examination revealed that the mass had further shrunk to axis lengths of 56 mm × 37 mm and exhibited the same enhancement pattern observed previously Fig. . BecauseMagnetic resonance imaging (MRI) performed in April 2015 demonstrated a tumor with isointensity to the spleen on T1-weighted images and slightly inhomogeneous hypointensity on T2-weighted images Fig. .A fourth CT examination performed in June 2015 revealed that the mass had further reduced to axis lengths of 49 mm × 34 mm Fig. . AlthougLaparoscopic partial gastrectomy with partial splenectomy was performed by an automatic suture. Operative findings revealed that the tumor was a hard mass and was firmly attached to the greater curvature of the stomach and the inferior pole of the spleen Fig. . It was Macroscopically, the tumor measured 60 mm × 50 mm × 25 mm, and the cut surface of the resected specimen was pink and uniform Fig. . MicroscThe patient’s postoperative course was uneventful and without complications. He continues to undergo surveillance for recurrence, and no signs of recurrence have been observed for 16 months after the operation.Desmoid tumors can be categorized based on three different localizations, the abdominal wall, intra-abdominal, and extra-abdominal, and the reported incidences of each type are 49, 8, and 43%, respectively. Intra-abdominal desmoid tumors are further classified into mesenteric and intrapelvic tumors. Desmoid tumors are also divided into FAP-associated and sporadic tumors. Although desmoid tumors can occur anywhere in the body, FAP-associated desmoid tumors are typically intra-abdominal. Moreover, almost all intra-abdominal desmoid tumors are associated with FAP and previous surgery. The incidence of desmoid tumors is approximately 10–15% among patients with FAP, and 12.3% of patients with desmoid tumors have been diagnosed with FAP [Given the aforementioned data, the present case was extremely rare because he had an intra-abdominal, sporadic desmoid tumor without a history of FAP, trauma, or surgery. In addition, it was particularly unusual that the patient’s tumor was derived from the stomach and gradually decreased in size. To the best of our knowledge, reports of desmoid tumors derived from the stomach and articles regarding spontaneous shrinkage of an intra-abdominal desmoid tumor are rather scant –12. ThusIn fact, despite its observed reduction in size over time, the tumor was neither a GIST nor a hematoma but rather a desmoid tumor. Diagnosis was difficult given the spontaneous decrease in tumor size. Few literature reports have described spontaneous shrinkage of desmoid tumors without treatment. A retrospective review has reported the disappearance or diminishing of five of eight tumors . The reaIntra-abdominal desmoid tumors have a tendency to recur locally after surgical resection, but they are not associated with the ability to metastasize . The recIt was difficult to regard the patient’s intra-abdominal tumor near the stomach as a potential desmoid tumor, in the described case involving a young boy without a history of FAP, trauma, or surgical procedures and with gradually shrinking intra-abdominal tumor. Thus, in such cases, it may be necessary to determine surgical procedures for certain diagnosis.


Sialadenoma papilliferum (SP) is a rare benign tumor, which originates from the minor salivary gland. It occurs at sites that have minor salivary glands, such as the palate, retromolar pads, buccal mucosa, and lips. The most common location for tumor development is on the hard palate. A differential diagnosis consists of ruling out other salivary gland tumors. Transoral robotic surgery (TORS) is a new technology used in head and neck surgery within certain centers around the world.Herein, we present the first successful case of SP tumor removal by TORS.This particular case highlights the identification of this rare tumor in an unusual location. Furthermore, it demonstrates the utilization of TORS, leveraging the superior visualization to obtain excellent local control with minimal acute and long-term morbidity, in comparison to conventional transoral surgical approaches.  First described by Abrams and Finck in 1969, Sialadenoma papilliferum (SP) is a rare benign tumor that often occurs at sites with minor salivary glands . Cases oA 72-year-old male smoker presented with progressive enlargement of a painful mass extending from the buccal mucosa to the mandıble corpus on his left retromolar pad. The mass had been growing for over 1 year and caused occasional bleeding. The patient’s history indicated a lack of any surgeries or chronic infection, but a history of smoking 65 packets of cigarettes per year. Oropharyngeal examination revealed the presence of an irregular, exophytic lesion in the left retromolar pad measuring approximately 3x3 cm, with a papillamatous hemorrhagic surface . Further otolaryngological and systemic examinations were unremarkable. Complete blood count (CBC), biochemistry, and coagulometer tests were applied. Incisional biopsy was performed because the patient was suspected of having a malignant salivary gland tumor. Computerized tomography (CT) and magnetic resonance imaging (MRI) was performed to reveal the relationship between bone structure and soft tissue within the oral cavity. All of the hematological test results were unremarkable. Histopathologically biopsy specimen showed dilated duct-like structures with an overliying squamous epithelium. The duct like structures were lined by double-triple layered epithelium with prominent papillary proliferations. The papillae showed thin fibrovascular cores. The result of the biopsy was consistent with SP . CT revealed the presence of a lytic lesion in the left mandibular corpus measuring approximately 19 mm . MRI revThe advantages and disavantages of TORS were explained to the patient. The patient chose to proceed with TORS, and subsequently underwent tumor excision via TORS with a laser-resistant nasotracheal tube under general anesthesia. The da Vinci robotic system was set up and docked with the patient cart. A Feyh-Kastenbauer retractor was used to expose the oropharynx. A 8 mm 0-degree da Vinci three-dimensional robotic camera was introduced into the oral cavity to visualize the operating field.Then two lateral arms were attached. For the TORS-SP, a 5 mm monopolar cautery device was attached to one arm, and a Maryland dissector was attached to the second arm. The tumor was completely removed with a 1 cm clear surgical margin, using a monopolar cautery device.The lytic lesion in left mandibular corpus was curetted completely and the defect in left mandibular corpus was primarily covered with oral mucosa. The Maryland dissector was replaced with a needle-driver, and surgical closure was performed using 2-0 vicryl . The total operation time was 30 min including a 5 min robot setup time. Total blood loss was limited to 10 cc during surgery. The patient started peroral liquid intake six h after the operation. Histopathologicaly the diagnosis was SP in the excision specimen. The result of curetted lytic bone lesion was consistent with SP. Postoperative complications and recurrence were not observed during the 1-year follow-up. Informed ConsentWritten informed consent was obtained from the patient described in this case. First described by Abrams and Finck in 1969, SP is an extremely rare benign tumor of the minor salivary gland ,3. AccorSP is a benign, exophytic, slow-growing, painless tumor of unknown origin. In the literature, there is inconstancy between the time the tumor is first noticed by the patient and when it is presented to the physician. The patient described in the study by Abrams and Finck ,4 had thSP is diagnosed by a correlation between clinical indications and histopathological examination. Microscopically, SP is characterized by an exophytic mass without encapsulation, and papillary proliferation of salivary gland ductal epithelium. This typically includes a double-layer of polyhedral and columnar cells with a concomitant overgrowth of squamous epithelium. A differential diagnosis of SP consists of eliminating the potential for several benign and malign salivary gland tumors such as mucoepidermoid carsinoma, papillary cystadenoma, adenolymphema, intraductal papillary tumors, Warthin’s tumor, and papillary squamous cell carcinoma ,5. ThereTreatment of SP involves local excision of the tumor, and many different surgical approaches currently exist ,6. In ouIn January 2010, the Food and Drug Administration approved TORS for use in benign and malignant disease of the tonsils, pharynx, and larynx . The useIn addition to the rarity of and little knowledge about this neoplasm, this case report exemplifies how TORS can be employed for excellent local control with minimal morbidity. The lytic bone lesion in SP of oral cavity is not contraindication for TORS. However, there is no enough knowledge about bone involvement and TORS for SP in literature.The prognosis of SP is excellent, and recurrence is not expected. Malignant transformation is very rare, but in literature some cases of mucoepidermoid carcinoma arising in a background of SP have been reported ,6. CloseThis case presented here highlights the identification of this rare tumor in an unusual location. Furthermore, it demonstrates the utilization of TORS, leveraging the superior visualization to obtain excellent local control with minimal acute and long-term morbidity in comparison to conventional transoral surgical approaches.


TSC1 or TSC2 genes  result in an increased activation of mTOR pathway. Optimal EAML treatment, including mTOR inhibitors, remains undetermined.Renal epithelioid angiomyolipomas (EAML) are rare tumors with aggressive behavior. EAML can be sporadic or develop within the tuberous sclerosis complex syndrome, where mutations of MTOR, TSC1 and TSC2 genes in the primary tumor, metastasis and blood of the patient, revealed one inactivating TSC2 mutation  in the tumor cells. Immunohistochemistry revealed decreased TSC2 protein content and increased phospho-S6 in the tumor cells, demonstrating mTOR pathway activation.Here we present the case of a young adult with a renal EAML that after radical nephrectomy developed metastases, first in liver and then in lumbar vertebrae. After complete surgical resection of these lesions, liver recurrence was detected, this time with incomplete surgical resection. After finding a new liver lesion, systemic treatment with sirolimus started. The patient exhibited a complete and durable response to this drug, being disease free at the time of publication, after 36 months of treatment. Targeted next generation sequencing (NGS) of TSC2 inactivation as the mechanism for the response. This study supports NGS as a useful tool to identify patients sensitive to mTOR inhibitors and supports the treatment of malignant EAML with these drugs.NGS on an EAML patient with an extraordinary response to sirolimus uncovered  Angiomyolipomas (AML) are rare kidney tumors that occur in 0.2–0.3% of the population , 2. ThesThe behaviour of EAML is variable, ranging from indolent, with only local growth, to aggressive, with potential for invasive growth and dissemination. This different behaviour seems to be determined by the presence or absence of cellular atypia and other clinical and pathological factors. The definition of atypical epithelioid cells in series of EAML includes atypical polygonal cells with abundant cytoplasm, vesicular nuclei, prominent nucleoli and nuclear size that exceeds twice the size of the adjacent nuclei. In a comparative analysis of the literature published by Brimo et al., 21 EAML cases with benign clinical course were compared with 9 EAML with malignant behaviour . The aggTSC1 or TSC2. These genes encode the proteins hamartin and tuberin responsible for the inhibition of the mammalian target of rapamycin (mTOR), a conserved protein kinase that regulates cell growth and metabolism in response to growth factors and nutrients [Approximately 80% of AML are sporadic while 20% develop within the TSC [utrients . Rapamycutrients . Signifiutrients , 12. Wheutrients –16.In this study we performed a genomic and immunohistochemical characterization of an EAML patient that after developing hepatic and bone metastasis had a complete response to sirolimus, and 36 months after the start of treatment remains disease-free. The molecular mechanism responsible for this extraordinary response to sirolimus was identified by next generation sequencing (NGS) and immunohistochemistry (IHC).TSC1 or TSC2 germline mutations detected).We report the case of a Caucasian male patient aged 34 without an irrelevant past medical history that presented with unexplained weight loss. Imaging studies revealed the presence of a left renal mass of 10 × 12 cm Fig. . The patMTOR, TSC1 and TSC2 genes was performed on DNA extracted from formalin-fixed paraffin-embedded primary tumor and hepatic metastasis, and the patient’s peripheral blood . Primary tumor failed NGS due to poor DNA quality, however, the liver metastasis and the blood were successfully sequenced by NGS, with a mean coverage of 184× and 1643×, respectively, and single nucleotide variants and indels were identified. One TSC2 variant resulting in a premature stop codon  was found in heterozygosity in the metastasis while it was absent in blood , not described previously in COSMIC or in the germline TCS2 Leiden Open Variation Database (LOVD). However, the LOVD includes a sporadic TSC patient with a TSC2 protein truncated at Threonine 913 (c.2737_2738delAC), allowing to classify this novel variant as pathogenic. Inactivation of TSC2 and over-activation of mTORC1 in the tumor cells were confirmed by IHC, and were in agreement with the extraordinary response to sirolimus.Genetic studies have shown that AML occur due to bi-allelic inactivation of either  or TSC1 . In the C1 [TSC2 , 23. In frequent , howeverTSC1/TSC2 mutations in sporadic AML and PEComas [Previous studies have reported mTOR pathway activation for  PEComas –27, sugg PEComas .However, therapeutic experience with aggressive EAML is scarce. There are only about a dozen published cases in the literature in which EAML patients received mTOR inhibitors with variable outcomes. These reports consist on clinical descriptions that include favorable responses, in most of the cases with no genetic study associated , 29–31. TSC2 deficiency in a sporadic EAML patient as the mutation causative of an exceptional response to sirolimus. These results support NGS as a useful tool to predict sensitivity to mTOR inhibitors in patients with EAML, this rare and potentially aggressive urological malignancy is misrepresented in clinical trials.In summary, only a few cases of EAML with benefit from mTOR inhibitors have been reported, and the mechanisms underlying these responses are unexplored. This study reveals


Adrenal hemangioma is a rare disease, with only some 60 cases reported previously. Due to the difficulty of the preoperative diagnosis of adrenal hemangioma, almost all of the cases were diagnosed by a histopathological analysis of surgical specimens.  A 52-year-old man was referred to our department for further examination of his left retroperitoneal tumor. He had received hemodialysis due to chronic renal failure resulting from membranous nephropathy. Computed tomography revealed a mass around his left hilum. Magnetic resonance imaging (MRI) and positron-emission tomography (PET)-CT were unable to confirm or deny malignancy, and tumor markers, including CEA and CA19-9, showed slight elevation. His tumor grew from 38 mm to 54 mm in diameter in 7 months of follow-up. We therefore planned retroperitoneal tumor resection with left nephrectomy. Histopathologically, hyperplastic small vessels with hemorrhaging and denaturation were seen. The endothelial cells showed no variants or division of the nucleus. Based on this diagnosis, no further therapy was performed. He has had no recurrence in the eight months since the surgery.  We herein report a rare case of adrenal hemangioma. Adrenal cystic disease is a rare entity, accounting for 2.3% of all adrenal tumors, and most such patients show no symptoms and are instead incidentally detected by computed tomography (CT) –3. Fosteμg/dL, aldosterone 410 pg/mL, adrenaline 19 pg/mL, noradrenaline 53 pg/mL, and dopamine 12 pg/mL. Tumor markers showed slight elevation of CEA, SCC, NSE, and IL-2 receptor as follows: CEA 5.7 ng/mL, AFP 7 ng/mL, CA125 27 U/mL, SCC 3.7 ng/mL, NSE 23.4 ng/mL, and IL-2 receptor 663.1 U/mL.A 52-year-old Asian man was referred to our department for further examination of his retroperitoneal tumor in April 2016. He had received hemodialysis due to grade 5 chronic kidney disease resulting from membranous nephropathy. His previous physician performed CT because the patient had lost 20 kg over the past 3 years, and CT revealed a left retroperitoneal mass. He had no remarkable physical findings. Laboratory data showed almost normal values, except for an elevated serum creatine level (4.31 mg/dL). A serum hormonal examination showed slight elevation in aldosterone as follows: cortisol 6.2 CT showed a retroperitoneal mass of 4.3 cm in diameter in April 2016. Because of the patient's low renal function, enhanced CT was not performed. Gd-enhanced magnetic resonance imaging showed low intensity on T1-weighted imaging (T1WI) and high intensity on T2WI . PositroThe patient underwent retroperitoneal tumor and renal resection and was discharged seven days postoperatively. The resected tumor was solid and dark blown in color, measuring 50 × 37 × 30 mm in size . HistoloTypical symptoms of adrenal hemangioma include pain, nausea, and vomiting, but 70%–80% of patients show no symptoms due to a lack of specific phenomena , 4. ReceTreatments for adrenal hemangioma in the previously reported cases included conservative observation and tumor resection. In cases with typical findings by CT or MRI, conservative therapy was performed. However, when imaging findings suggested adrenal hemangioma, surgical treatment was selected for larger tumors because of the difficulty of a preoperative diagnosis. Grumbach and Yamakita reported the indications of surgical resection. Grumbach et al. suggested surgical resection be performed for adrenal tumors >6 cm in diameter, given the 25% incidence of malignant diseases; no apparent indications have been established for tumors 4 to 6 cm in size . YamakitIn our case, although the tumor size was 43 mm, careful observation was selected due to suspicion of adrenal hemangioma based on the CT and MRI findings.Nakagawa and Fuzimoto reported that, in cases of a large tumor size, adrenal hemangioma accompanied malignant tumor  , 13. TheWe herein report a case of adrenal hemangioma that was difficult to diagnose preoperatively. Due to the enlargement of the tumor during follow-up, the patient had to undergo tumorectomy.


We present a rare case of renal gastrinoma. To the best of our knowledge, only one case of renal gastrinoma has been reported in the literature so far. An African American male was diagnosed with Zollinger Ellison syndrome at the age of 15 years, when he underwent surgery for peritonitis secondary to duodenal ulcer perforation. Further evaluation was deferred and proton pump inhibitors were prescribed. Later evaluation showed a left renal mass. Serum gastrin levels were 4,307 pg/ml. A CAT scan of the abdomen showed 4- x 4-cm heterogeneous solid mass in the interpolar region of the left kidney with central hypodensity. Somatostatin scintigraphy confirmed a receptor-positive mass in the same location. Nephrectomy was done and the tumor was diagnosed on histopathological examination as a gastrinoma. At 6-month follow-up, gastrin levels were 72 pg/ml. After a follow-up of 6 years, the patient has no recurrent symptoms.


Primary retroperitoneal serous cystadenomas (PRSCs) are rare cystic lesions whose pathogenesis is currently not well understood. Although the vast majority of tumors are benign, early recognition and resection is necessary to avoid malignant transformation, rupture, and secondary infection. Here we present the case of a 79-year-old woman who presented with confusion, visual hallucinations, and a history of fall. As part of the work-up for abdominal distension, computed tomography scan of the abdomen and pelvis was performed, which revealed a right-sided retroperitoneal cystic lesion measuring 26.6 × 16.7 cm in size. The lesion was resected laparoscopically, and the surgical specimen measured 28 × 17 cm. Histology revealed a serous cystadenoma. The postsurgical course was uneventful, and no radiological recurrence was noted on 3 months follow-up. Very few primary retroperitoneal cystic lesions have been reported in the literature. Most lesions are benign and predominantly occur in females. They may remain asymptomatic for long periods of time and are usually discovered when they reach very large in size. In rare cases, these lesions may have malignant potential. Diagnosis of PRSC should be considered in the differential diagnosis of all retroperitoneal cysts. Primary retroperitoneal serous cystadenoma (PRSC) is an extremely uncommon lesion of retroperitoneum. Very few case reports of this entity have been described in the literature. The pathogenesis is not well understood, although one of the proposed hypotheses considers it to be an embryological remnant of the urogenital apparatus with epithelial and mesothelial tissues.A 79-year-old female presented to the emergency room with confusion, agitation, and visual hallucinations after sustaining a fall. Although the patient was a poor historian, her family members did not report prior history of abdominal complaints including trauma, fever, weight loss, abdominal mass, or surgery. Abdominal examination revealed soft, nontender, palpable fullness in the paraumbilical region. Gastroenterology service was consulted for a nontender abdominal fullness on physical examination as well as laboratory findings of severe iron deficiency anemia. The rest of her laboratory findings were unremarkable.A supine and upright abdominal film showed bowel loops displaced to the left with a large amount of stool in the colon . A noncThe patient was taken to the operating room with a high suspicion that the CT findings were consistent with a giant liver cyst. Upon entering the abdomen using a Hasson technique and placing a 12-mm trocar, a diagnostic laparoscopy revealed a large cyst with a blue hue. Given that the CT findings showed no evidence of nodularity, septation, or intracystic masses and the laparoscopic findings were consistent, the suspicion of malignancy was very low. Upon drainage of the cyst, it was clearly noted that it was easily separable from the posterior liver and was in fact retroperitoneal in origin. The posterior peritoneum was incised. The wall of the cyst was carefully dissected from the anterolateral wall of the kidney, adrenal, and the posterior wall of the ascending colon keeping in mind the significant distortions in anatomy secondary to its size. A plane between the cyst and the posterior peritoneum was established, and the cyst was separated using a combination of electrocautery and sharp dissection. The cyst was then placed in an endocatch bag and removed through the 12-mm port. Thus, the patient underwent a complete cyst excision using a laparoscopic transperitoneal approach. Intraoperatively, the retroperitoneal location of the cyst was confirmed as the cyst wall was separated from visceral peritoneum with ease . MacrosThe retroperitoneal space is bounded anteriorly by the posterior part of parietal peritoneum, posteriorly by the psoas and quadratus lumborum muscles, as well as the spine, superiorly by the diaphragm, and inferiorly by the muscular wall of the pelvis.Primary retroperitoneal cystic lesions are extremely rare because of a lack of epithelial cells in this region. Their incidence is difficult to estimate. The pathogenesis of primary RPCs is not well understood. Several hypotheses are described in the literature, such as: (1) coelomic epithelial metaplasia,Clinical presentation of retroperitoneal cystic lesions may vary depending on their location and size. Most commonly, cysts present as a palpable mass or abdominal pain. Constitutional symptoms such as fever, changes in appetite, and weight loss may be present especially in malignant lesions. Our patient had a palpable mass on examination, leading to the further work-up and diagnosis.There are no pathognomonic signs, symptoms, and laboratory or imaging findings reported in the literature to confirm the diagnosis. However, few case reports suggest association with elevated blood levels of carcinoembryonic antigen (CEA), fetoprotein, CA125, CA19-9, and CA15-3.PRSC should be differentiated from other forms of cystic retroperitoneal lesions based on history and microscopic appearance, as well as its malignant form cystadenocarcinoma . Lee atDiagnostic fluid aspiration is discouraged due to concerns of seeding during the procedure if the lesion is malignant. The treatment of choice is complete surgical excision. The type of surgical intervention depends on the location, size, and expertise of the surgeon. Historically, laparotomy and complete cyst enucleation has been the recommended treatment approach. However, laparoscopic cyst excision is becoming more popular in recent years. Marsupialization and partial excision of the cysts or fluid drainage are avoided given higher risks for recurrence as well as the need for repeat surgical procedures. Obscure walls and location in close proximity of major blood vessels and organs are the main challenges during RPC excision. Recurrence can occur in approximately 25% of cases of RPCs according to a case series.PRSCs are extremely rare lesions and oftentimes remain asymptomatic until they attain a very large size. Although advances in CT and MRI techniques enable us to identify various cystic lesions of the retroperitoneum, the exact diagnosis is based on histology and requires high clinical suspicion as well as expertise. We believe that endoscopic ultrasound may have a promising role in diagnosis, although further large prospective studies are required to confirm that. All attempts should be made to completely remove the cysts as risk of recurrence is high in partially excised lesions. Malignant lesions should be treated more aggressively with radical surgery and chemotherapy. More research is needed to better understand the pathogenesis of this entity.


Mycobacterium tuberculosis. The patient had a complete remission after the use of antituberculosis drugs.Pelvic tuberculosis is a type of extrapulmonary tuberculosis. The disease is accompanied by clinical and laboratory findings which may be unspecific and present aspects of other diseases, including gynecological malignancies. In this report, the authors presented a case of pelvic tuberculosis associated with peritoneal tuberculosis in a young woman exhibiting imaging and tumor markers consistent with ovarian neoplasm. An intraoperative frozen section analysis detected atypical cells that were suggestive of ovarian borderline or malignant epithelial neoplasia. The pathological analysis showed granulomatous inflammation in the right ovary and fallopian tube with a pattern of mycobacteriosis that was consistent with the presence of mycobacteria morphologically compatible with Ovarian cancer cases are often characterized by the presence of ascites, adnexal masses, and high CA-125 levels. Radical surgery is generally the primary treatment, and this can include removal of the uterus, ovaries, fallopian tubes, and pelvic lymph nodes [ Mycobacterium tuberculosis [Pelvic tuberculosis is a type of extrapulmonary tuberculosis that is caused byrculosis , 4. The rculosis , 6. Howerculosis . Risk farculosis , 7.Here, we report a case of pelvic tuberculosis associated with peritoneal tuberculosis in a young woman exhibiting symptoms consistent with ovarian cancer.A 24-year-old woman presented to our Pelvic Mass Ambulatory Unit ) with a history of abdominal pain for the previous week that was associated with weight loss, fever, diarrhea, nausea, and ascites. A chest X-ray was normal. Abdominal ultrasonography showed an enlarged right ovary , with heterogeneous echogenicity, cystic areas, and thick walls. Magnetic resonance imaging (MRI) showed a heterogeneous mass with well-defined edges involving the right ovary that measured approximately 5.0 cm in diameter and exhibited heterogeneous contrast enhancement. The left ovary and uterus were normal. Additional observations included enlarged iliac lymph nodes, smaller lesions in the posterior fornix, abdominal ascites, and diffuse enhancement of the peritoneum. CA-125 and CA-15.3 levels were also increased .An exploratory laparotomy was performed. The pelvis was frozen and the entire bowel and omentum were congested, similar to peritoneal carcinomatosis. During the procedure, biopsies were performed of the pelvic peritoneum, the fallopian tube, and the right ovary. An ascite sample was also collected. An intraoperative frozen section analysis detected atypical cells that were suggestive of ovarian borderline or malignant epithelial neoplasia . Despite Mycobacterium tuberculosis.The pathological analysis showed granulomatous inflammation in the right ovary and fallopian tube with a pattern of mycobacteriosis that was consistent with the presence of mycobacteria morphologically compatible withThe patient was referred to the Infectious Diseases Service and received antituberculosis drugs. Six months later, the patient reported overall good health. Her CA-125 levels and an abdominopelvic ultrasound were both normal. Upon completion of the tuberculosis treatment, the patient was trying to become pregnant.Pelvic tuberculosis can present with nonspecific symptoms and similar laboratory and clinical findings to ovarian malignancy. Here, a patient with pelvic tuberculosis experienced pain and weight loss that were accompanied by increased tumor marker levels, the presence of ascites, and an ovarian mass. Surgery was necessary to confirm a diagnosis and treatment plan.The presence of abdominopelvic pain, ascites, nausea, vomiting, and anorexia associated with imaging tests showing pelvic mass were reported in patients with abdominopelvic tuberculosis , 8–11 anThe treatment of pelvic tuberculosis is usually nonsurgical. It is difficult to diagnose pelvic tuberculosis prior to surgery, and currently there are no laboratory tests or imaging modalities that can differentiate this disease from advanced ovarian cancer . MoreoveIt is important to note that pelvic and pulmonary tuberculosis are not always associated. A standard chest X-ray is often performed, and computed tomography of the abdomen and pelvis can show ascites and induration of the mesentery and omentum . CA 125 A BAAR analysis of ascites also has a low sensitivity for a diagnosis of peritoneal tuberculosis. However, an advance in the analysis of ascitic fluid in this context has been the detection of adenosine deaminase (ADA). This enzyme is involved in the conversion of adenosine to inosine and is released by macrophages and lymphocytes during an immune response. In a previous study it was demonstrated that a cutoff level of 39 U/I for ADA levels provided high sensitivity and specificity for a diagnosis of abdominopelvic tuberculosis . FurtherLaparoscopy as a diagnostic tool in combination with biopsy and histology was found to be effective for establishing a diagnosis of pelvic tuberculosis. Laparoscopic biopsy is a sensitive and specific diagnostic procedure for abdominal peritonitis, and it is considered to be the gold standard method for clearly differentiating advanced ovarian cancer and peritoneal tuberculosis, especially in areas where tuberculosis is endemic . HoweverAlthough frozen section has good accuracy for malignant and benign neoplasms, its efficiency in detecting borderline ovarian tumors is not high, perhaps because more extensive samples are needed , 20. IntThe ability to provide a preoperative differential diagnosis of pelvic tuberculosis that mimics an ovarian tumor remains a challenge, especially in the absence of specific protocols, nonspecific symptoms, and the rarity of cases that characterize this disease. As demonstrated in the present case, conservative surgery should be performed when a diagnosis is uncertain, especially for patients who wish to become pregnant.


Diaphragmatic hernia following blunt abdominal injury is extremely rare and often diagnosed late. Missed diagnosis is also common with this condition. We herein present a delayed presentation of diaphragmatic hernia following blunt abdominal injury that was initially misdiagnosed as recurrent acute asthmatic attack due to repeated presentation with episodic difficulty in breathing. Blunt traumatic rupture of the diaphragm is a serious injury that is often difficult to diagnose . As manyDiaphragmatic hernia following blunt abdominal injury is extremely rare and occurs in 0.8–1.6% of cases . It is uWe herein present a case of delayed presentation of diaphragmatic hernia masquerading as recurrent asthmatic attack two years after sustaining blunt abdominal injury.A 40-year-old business man presented to general surgery outpatient clinic with one-and-half-year history of recurrent difficulty in breathing and chest tightness. He had no history of cough, orthopnea, paroxysmal nocturnal dyspnoea, body swellings, or fever and no gastrointestinal symptoms. On account of this, he was treated on several occasions in various hospitals as acute asthmatic attack with no relief of his symptoms. He had past history of road traffic accident with blunt abdominal injury and head injury 2 years prior to presentation that was managed conservatively. There was no other significant medical history.On physical examination, he was found to be well-preserved young man. He had a reduced air entry on lower left hemithorax with audible bowel sounds on the same site of the chest.A chest X-ray shows obscured left hemidiaphragm, presence of gastric fundal gas, and air fluid level in left hemithorax . Barium He had laparotomy with intraoperative findings of 15 cm left diaphragmatic defect , with hePatients with posttraumatic diaphragmatic herniation frequently present months to years after the initial injury. The proposed reason for the delay in symptoms may be the presence of omentum and viscera plugging the diaphragmatic defect temporarily, allowing for symptomatic visceral herniation to occur months to years later . Missed Previously, Edino and colleagues have reported a case of gastropleurocutaneous fistula in a patient with delayed diagnosis of left diaphragmatic hernia .Chest X-ray is the initial investigation of choice in patients with suspected diaphragmatic hernia and has sensitivity of 30–62% . RadiogrOther plain radiograph findings that may suggest possibility of diaphragmatic rupture include abnormalities such as multiple lower rib fractures, haemothorax, and pneumoperitoneum . OccasioGastrointestinal contrast study may reveal evidence of bowel herniation into the thoracic cavity further increasing the diagnostic accuracy .Our patient had herniation of the transverse colon on barium enema investigation. Others include upper gastrointestinal contrast studies, diagnostic peritoneal lavage, fluoroscopy, ultrasound, magnetic resonance imaging, laparoscopy, and intraperitoneal injection of radioisotopes .Treatment of diaphragmatic hernia is surgical via laparotomy or thoracotomy. Acute cases are better managed via a laparotomy as this also rules out and treats associated intra-abdominal organ injuries. Delayed cases, however, are better treated via a thoracotomy or thoracoabdominal approach because of intrathoracic adhesions , 11. OurDespite the fact that diaphragmatic hernia following blunt abdominal injury is rare, it should be suspected in a patient presenting with recurrent difficulty in breathing with previous history of blunt abdominal injury.


Chondrosarcoma is considered as a common primary bone sarcoma. These sarcomas can form large masses without any specific symptoms because there are no barriers in pelvic anatomy to prevent the enlargement of tumors, and can mimic ovarian masses. We present a pelvic chondrosarcoma in a woman aged 37 years who was misdiagnosed as having an ovarian mass due to the limited information obtained from imaging studies. Pelvic chondrosarcoma should be considered in patients who have pelvic masses with solid components. It should be kept in mind that interventions should be performed at centers where there are orthopedic surgeons with experience of this subject. Moreover, this tumor arises predominantly after the second decade with a peak in the middle-aged period. Many heterogeneous groups of neoplasms are related to chondrosarcoma and cartilage matrix production is a typical feature of this tumor. These sarcomas form large masses without any specific symptoms because there are no barriers in the pelvic anatomy to prevent the enlargement of tumors. In this case report, we present a patient who had a tumor of undefined origin according to preoperative imaging methods, and a pelvic chondrosarcoma mimicking an adnexal mass. To the best of our knowledge, this is only the third case of pelvic chondrosarcoma in the English literature of obstetrics and gynecology.Chondrosarcoma is considered as a common primary bone sarcoma, which is ranked as the third primary bone malignancy. One in five cases of bone sarcomas are due to chondrosarcoma, and half of all cases affect the pelvisA 37-year-old single woman presented to our clinic with symptoms of pelvic pain, abdominal distension, and vaginal bleeding. During the pelvic examination, a mass that filled the pelvis was detected. The carcinoma antigen 125 (CA125) level was 40 U/mL. Ultrasonographic and magnetic resonance imaging (MRI) examinations revealed a solid mass 20x10 cm in diameter including calcified areas, and a mass consistent with myoma of 3.5 cm in diameter was observed in the cervico-isthmic region of the uterus. The endometrial thickness was 8 mm . During . After reviewing all operated cases of pelvic mass in our department within the last 15 years, chondrosarcoma was the only case that was misdiagnosed as an ovarian mass, which reflects the sporadic incidence of this condition. Although all available imaging techniques, even CT and MRI, were used to assist us in mapping this tumor, we could not identify the exact margins, neither the exact organ from which this tumor derived. Moreover, the elevated CA125 was another misleading factor that increased our suspicion of ovarian mass. The surgical management of chondrosarcoma is a destructive operation for orthopedic surgeons due to the following principles: increased risk of vital organ injuries, high susceptibility to damage pelvic structural stability, challenging anatomic interactions of the pelvis, and devastating extension of the tumor. The mass could not be completely removed and the patient was considered as non-resectable and referred to another orthopedics clinic because we did not have an orthopedic surgical team skilled in pelvic chondrosarcoma. Vast majority of chondrosarcoma become symptomatic after reaching a large size and this can be explained by the slow growth rate of this these tumors. Grade 1 chondrosarcomas consist of profuse hyaline cartilage matrix surrounding a little cellular mass and metastasize infrequently. In our case, the pathologic result was grade 1 chondrosarcoma, and there were metastatic lesions observed in the liver in abdominal tomography. Pelvic chondrosarcomas can appear in various pathologic neoplasms, other than pure chondrosarcoma arising from the pelvic bone, such as chondrogenic tumors of the ovaries or heterologous carcinosarcomas of the uterus. However, all mentioned types have very low incidence rates. Moreover, mixed mesodermal tumors, commonly called carcinosarcoma, another manifestation of chondrosarcoma, are the most common heterologous sarcomas, originating from the uterus in most cases. The histologic appearance is a mixture of ectoderm and mesoderm-derived tissues. Either homologous or heterologous mesodermal tissue can be commonly found to be high grade. Heterologous tumors consist of a differentiated mesenchymal component accompanied by endometrial, stromal or undifferentiated sarcomas. Occasionally, these uterine tumors may enlarge and convert to giant pelvic masses that can destroy the uterine structure to the point where it can become unrecognizable. The cornerstone of treatment in managing this tumor is wide surgical excision, that still first line treatment. After performing extensive intralesional curettage, local adjuvant treatment has encouraging long-term outcomes and adequate control of local recurrence in low-grade chondrosarcomas. Local adjuvant treatment is only effective in cases with well-defined boundaries with no extension beyond the bone. However, giant tumor or pelvic localization of chondrosarcoma can alter the treatment method, even in low-grade conditions, as in our case, which require wide resection as a first-line management, rather than intralesional curettage.That a pelvic chondrosarcoma could masquerade as an ovarian mass was considered the peculiar part of our case. This camouflage exposes the limited role of imaging techniques and tumor markers in diagnosing pelvic tumors. Generally, chondrosarcoma has a silent course due to the special pelvic structure, which allows masses to grow feasibly without any boundaries and only become symptomatic after enlarging enough, as in our case. Thus, this tumor has a larger size in comparison with other pelvic masses when the diagnosis was established; the mean size is commonly 11 cmPelvic chondrosarcoma should be considered in patients who have pelvic masses with solid components because preoperative evaluations  in daily practice may be inadequate for the diagnosis of pelvic masses. As a consequence, it should be kept in mind that interventions should be performed at centers where there are orthopedic surgeons with experience of this subject.


Schwannomas of gastrointestinal tracts are rare and difficult to detect preoperatively because of negative results of endoscopic and imaging examinations. Here, we reported a case of rectal schwannoma, which was diagnosed by immunohistochemical staining after laparoscopic protectomy.A 61-year-old woman complained of a 1-month history of difficulty in defecation and irregularly abdominal discomfort during her physical checkup in our hospital.Immunohistochemical staining results after laparoscopic protectomy revealed a strong positive reaction for S-100 protein. Therefore, rectal schwannoma was confirmed.Treatment with laparoscopic protectomy was given.Symptoms resolved completely after 12 days of the surgery, and was regular followed-up in outpatient clinic.Schwannomas are difficult to identify preoperatively, and immunohistochemical staining for S-100 protein is an effective method to diagnose it. It has been considered as a minimally invasive treatment for colorectal diseases. Here, we present a case of rectal schwannoma in a 61-year-old woman treated by laparoscopic surgery.Schwannnoma is a tumor originating from the schwann cells, which forms the nerve sheaths. Schwann cells commonly grows along the peripheral nerves of the head and cervix, so schwannomas of gastrointestinal tracts are extremely rare.2This study was approved by the Ethics Committee and institutional review board of the Second Hospital of Jilin University, Changchun, China. Written informed consent was obtained from the patient for publication of this report.A 61-year-old woman complained of a 1-month history of difficulty in defecation and frequently abdominal irregular discomfort during her physical checkup in our hospital. She had a 10-year history of well-controlled hypertension in her medical history. Physical examination showed no remarkable signs on abdomen but a firm mass with intact mucosa at 12 o’clock of the rectal wall detected via digital rectal examination. Colonoscopy revealed a tumorous lesion, which measured 4 cm in diameter, 6 cm from the anal verge, located in the rectum. The surface of the mucosa covering the lesion was intact , and desmin. Furthermore, a diagnosis of tumor malignancy is based on the status of the Ki-67 labeling index (MIB-1), whereby a highly reproducible immunopositivity for MIB-1 is considered as an indicator of malignancy. In this case, immunohistochemical findings include these criteria. Thus, we diagnosed it as benign schwannoma.Literally, histopathological observations by hematoxylin–eosin staining of tumor tissue have shown that schwannomas are composed of elongated bipolar spindle cells with zonally variable cellularity and focally prominent nuclear palisading pattern. However, relapse is not uncommon. Development of cysts relapses up to 15% with the development of disease, approximately 9% to 45% of rectal malignant tumors are commonly solid than cystic, which has a recurrence rates of 45%, and its 5-year survival rate is merely about 8% to 17%. In our case, no evidence of aggressive behavior was found based on 3-month postoperative follow-up information, which indicated that benign schwannomas have a good prognosis. However, a long-term clinical follow-up should be given to monitor the possible malignancy.Schwannoma is a kind of benign tumor, the survival rate of it approaches to 100%.In conclusion, rectal schwannomas are extremely rare and only few cases have been reported; immunohistochemical staining for S-100 protein is an effective method for differential diagnosis; and schwannomas are frequently diagnosed as benign tumors and carry a good prognosis.


We present an unusual case of a 41-year-old male patient with a large lymphangioma of the esophagus. Endoscopy revealed that the structure measured 60 × 10 mm in the mucosa and the submucosa and had a heterogenous echo pattern. The esophageal mass was successfully resected by endoscopic piecemeal mucosal resection. However, most esophageal lymphangiomas that are larger than 2 cm in diameter reported in the literature can be removed only through open surgery. Thus far, we know of no reported cases of endoscopic resection as a treatment for this case. Lymphangioma of the esophagus is a benign cystic tumor, which is characterized by the presence of chylous or serous fluid within an irregularly dilated lymphatic channel. To date, it has been a rare clinical entity. Most esophageal lymphangiomas reported in the literature are smaller than 2 cm in diameter and can be removed using endoscopy. The present report describes a patient with lymphangioma of the esophagus. Endoscopy after admission revealed a 60 × 10 mm mass in the esophagus, which was then treated by endoscopic piecemeal mucosal resection (EPMR).A 41-year-old man was admitted to the neighborhood hospital because of dysphagia when eating for the past three months. The gastroscopy showed a submucosal mass in the distal esophagus. He was then transferred to our hospital on August 4, 2011, for further examination and treatment. The personal medical and family histories were unremarkable. There was no abdominal pain, nausea, vomiting, hematemesis, melena, or weight loss, and the physical examination and standard laboratory tests were within normal limits.The endoscopy after admission revealed a large, whitish-yellow, translucent mass with a lustrous surface in the mid and distal esophagus located 32 to 38 cm from the incisors . The mucContrast-enhanced CT of the esophagus showed the lesion located in the lower esophagus Figures . The esoLymphangiomas are benign cystic tumors. They are characterized by the presence of chylous or serous fluid within an irregularly dilated lymphatic channel. Lymphangiomas may appear in any organ except the brain, although they commonly develop in the head and neck region, and only 1% are found in the GI tract. Of those that arise in the gut, the majority are found in the colon, followed in order of frequency by the stomach, duodenum, small intestine, and esophagus . They arNo cases of malignant transformation have been described because lymphangioma is a benign tumor , and gasOur treatment has demonstrated that it is feasible and safe to enucleate lymphangioma even for large lesions. To the best of our knowledge, the endoscopic resection has the advantages of a short operation time, fewer injuries, and a rapid recovery. We believe that EPMR is a safe and sufficient method of therapy for esophageal lymphangioma lesions less than 5 cm in diameter and those located in the mucosa and submucosa.1. Plain scan CT. Plain scan CT of the esophagus showed the lesion located in the lower esophagus. The CT value is 16 Hu.2. Enhanced CT. Enhanced CT of the esophagus showed the lesion located in the lower esophagus. The CT value is 38 Hu.3. EUS. A large, whitish-yellow, translucent mass with a lustrous surface in the mid and distal esophagus, located 32 to 38 cm from the incisors. A honeycomb-like hypoechoic structure measuring 60 × 10 mm located in the submucosa with heterogenous echo pattern; the muscularis propria was intact.4. EPMR. The esophageal mass was resected by endoscopic piecemeal mucosal resection (EPMR).5. Pathology. Histology of the resected mass showing cystically dilated lymphatics in the surface squamous epithelium and in the submucosa. .


We report a 21-year old female patient who presented with an 18-month history of a swelling in the distal phalanx of her right little finger. Although the history, clinical features and MRI were suggestive of a benign tumour or a tuberculous lesion, the histo-pathological examination of the swelling was reported as a conventional osteosarcoma. Osteosarcoma of the hand is very rare. This article highlights the possibility of a seemingly benign lesion seen in a routine clinic could well turn out to be malignant, and the need to maintain a high index of suspicion. The incidence of osteosarcoma of the hand has been reported to be between 0.18% – 0.39% of all skeletal osteosarcomas2. We report a rare presentation of osteosarcoma of the distal phalanx and its diagnosis and treatment.Osteosarcoma is the most common primary bone sarcoma typically originating from the metaphysis of long bones. It has a peak incidence at the age of 15-19 years with a high tendency for systemic spreadA 21-year old Vietnamese female patient presented with an eighteen-month history of a right little finger swelling associated with increasing pain. She had no fever, loss of weight or loss of appetite.2 on the dorso-lateral aspect of the distal phalanx of the right little finger . Pain and swelling were the most common presentations similar to our patient. There was a predilection for the metacarpo-phalangeal joints with the second metacarpal head being the commonest site of involvement. This correlates with the most active bone growth site in the hand.Fowble in 2005, reviewed 41 cases of osteosarcoma of the hand reported in 39 patients4 reviewed 123 cases of metastatic tumours of the hand and reported the more distal sites  were more often involved. Our patient’s presentation of idiopathic osteosarcoma arising solely in the distal phalanx is rare. There was a possibility that this could be a multifocal or multicentric osteosarcoma. However, our patient declined further investigations, hence we were unable to detect a possible primary tumour elsewhere. A bone scan in our patient may have allowed us to elucidate the cause of her terminal phalanx involvement.On the contrary, KerinThere are no known associated patient risk factors for developing this rare tumour.Conventional osteosarcoma is a grade by itself denoting a high-grade tumour.The usual MRI findings in osteosarcoma of tubular bones are similar to those of long bones with aggressive lesions, cortical destruction and periosteal reaction, together with abnormal marrow signal intensity and peritumoral edema. It was the absence of these features in our patient’s MRI, which led us to a conclusion of a benign lesion. Osteosarcoma and tuberculosis have similar radiographic findings and extra vigilance with tuberculosis is in order as it is well known as the great mimic.5 In our patient, a definitive wide margin was achieved . Retrospectively, a high index of suspicion for osteosarcoma is needed for accurate diagnosis and prompt and proper treatment.Evaluation and treatment of osteosarcoma of the phalanx should be similar to osteosarcoma of the long bones. Evaluation and treatment of conventional osteosarcoma of the fingers is amputation similar to long bones. Salvage was not an option as we had unknowingly performed curettage on a malignant lesion thus preventing us from obtaining clear tumour margins for such procedures. Chemotherapy and staging is indicated for osteosarcoma but the patient declined due to financial issues. Patients require CT evaluation of the thorax and abdomen and a bone scan, followed by the appropriate surgery and chemotherapy. Those with wide and radical margins had no local recurrence, but those with marginal excision had a considerably high recurrence rateWe missed the diagnosis of osteosarcoma as our initial diagnosis of tuberculosis was based on the benign features of the patient’s physical examination findings and the MRI report and the increased risk of having come from a tuberculosis endemic area. A constant awareness for unusual lesions in unusual locations is important so that proper and prompt treatment can be provided for our patients.


A three-month-old female infant presented with abdominal distention for 2 months. A large palpable mass in right upper quadrant was noted on physical exam. Abdominal ultrasound revealed a large heterogeneous mass with multiple cystic components. Mass was surgically excised and pathology was consistent with mature adrenal teratoma. Teratoma is a germ cell tumor mainly found in gonadal tissues. Occurrence of adrenal gland teratoma in children is very rare with less than 10 pediatric case reports in English literature. We present a rare case of primary adrenal tumor in an infant and a review of the literature.  Teratoma is a rare germ cell tumor (GCT) that develops from totipotent stem cells which differentiate into chaotically intermixed tissues that are foreign to the anatomic site in form which they arose. Tissue components that comprise the tumor are from all three embryonic germ layers; endoderm, mesoderm, and ectoderm . IncidenA three-month-old female infant, without any significant past medical or perinatal history, presented with a 2-month history of a gradually increasing abdominal distention. Patient was otherwise growing and developing well. A large palpable mass was found on examination which was predominantly located in right upper quadrant, cystic in consistency. An abdominal ultrasound was performed and revealed a large heterogeneous mass (8 cm × 10 cm) with multiple cystic components and calcification with inferior displacement of right kidney. An abdominal magnetic resonance imaging revealed a heterogeneous mass extending from the right suprarenal fossa and into the peritoneal cavity measuring 10.2 cm × 10.5 cm × 8.5 cm with multiple large collections consistent with cystic components, displacing the right kidney inferiorly .To rule out neuroblastoma MIBG scan was performed and was negative and urine vanillylmandelic acids (VMA) were also negative. Additionally other tumor markers, alpha-fetoprotein (AFP), human chorionic gonadotropin (hCG), and LDH, were appropriate for age. Complete blood counts were also normal including hemoglobin and hematocrit.The patient underwent open laparotomy. The mass was located in the right retroperitoneum pushing right colon, duodenum, IVC, portal vein, pancreas, and portal triad anteromedially and liver and gall bladder superiorly. No metastatic disease was identified. The mass was completely excised. Gross dimensions of the tumor were 10 cm × 10 cm × 8 cm, weighing 380 grams. The mass had heterogeneous quality with multiple cystic areas as well as hard bony areas. It consists of an unoriented, irregular, partially opened tan-white mass. Cut section of the mass revealed cystic spaces and solid areas. Cystic spaces were filled with clear fluid and lined with smooth tan/pink surface with focal areas of hemorrhage . The tumTeratomas are classified as 1 of 4 variants: mature, immature, teratoma with malignant transformation, and monodermal . AccordiAdrenal teratomas are usually benign, have no clinical manifestations, and are not associated with elevated markers unless malignancy is present. The retroperitoneum provides a large space for the mass to grow; thus tumors are typically large at the time of presentation. Patients can present with abdominal distension, abdominal pain, low back pain, and intestinal obstruction caused by compression or may be asymptomatic and the mass can be discovered incidentally. Cystic teratomas may rupture and cause sudden onset of abdominal pain, ascites, and peritonitis , 14.The diagnosis of adrenal teratoma relies predominantly on imaging because the findings from laboratory examinations will often be normal. Plain abdominal film often shows calcification. Sonography can identify the cystic, solid, or complex components of the tumor . Magneti


Elastofibroma is a benign tumor of the musculoskeletal system characterized by the abnormal accumulation of elastinophilic fibers. It has been classically described for subscapular region but has been reported in several musculoskeletal sites over the years and rarely even in the GI tract but never in pancreas. We therefore present the case of a 45-year-old female who presented with intermittent abdominal pain. CT of abdomen revealed 1.4 cm pancreatic neck lesion without peripancreatic lymphadenopathy. Endoscopic ultrasound (EUS) guided FNA was nondiagnostic. Surgical resection was performed with central pancreatectomy. Histopathology revealed well demarcated nodules of hypocellular collagen with abundant elastic fibers, characteristic of pancreatic elastofibroma. Treatment is not needed unless symptomatic and surgical resection is the preferred therapeutic option when indicated. This case adds another entity to the differential diagnosis of pancreatic mass lesions. Elastofibroma is a benign slow growing soft tissue tumor composed of characteristic elastinophilic fibers. According to WHO classification, it is currently considered to be a member of benign fibroblastic or myofibroblastic soft tissue tumors found in the periscapular region . It was A 45-year-old female with history of joint pains  presented with intermittent abdominal pain of few years' duration. General physical exam and serum chemistries were unremarkable. CT scan of her abdomen revealed a 1.4 by 1.4 cm hypodense lesion in the neck of pancreas just anterior to portal confluence, with no vascular involvement. Another 6 mm nodule thought to represent either a lobulation of the larger mass or a new lesion was also seen adjacent to it. No peripancreatic lymphadenopathy was apparent. Subsequently, an endoscopic ultrasound (EUS) was done. It showed a 2.5 × 1.2 cm hypoechoic mass in the pancreatic neck region . FNA of Elastofibroma is a relatively uncommon benign tumor of the musculoskeletal system. Elastofibroma has a predilection for subscapular area and is more commonly found in elderly females. It is characterized by abnormal accumulation of elastin fibers, pathogenesis of which is unclear . SeveralOver the years it has been reported at several locations throughout the musculoskeletal system including foot, spine, deltoid, greater trochanter, chest wall, and face , 10–12. Despite the uncertain pathogenesis, treatment is usually not recommended unless it is symptomatic. Most of the literature is in the form of case reports but surgical resection is the preferred therapeutic option when definitive treatment is required , 15, 16.


Sparganosis is one of the neglected but important food-borne parasitic zoonoses, with higher prevalence in Asian countries. The infection is commonly located in the subcutaneous tissue, brain, breast, and lung, but fewer reported infections involve the eye. Because the majority of patients with sparganosis are adults, it is likely to be missed in children.An 8-year-old boy presented to our clinic complaining of a painless ocular mass in his right eye for 1 month. The boy had a history of eating frogs and frog poultice applications to his eyelids. The patient was checked for an elliptical mass near the medial wall of the right eye. Serodiagnosis testing was positive in an enzyme-linked immunosorbent assay. During surgical operation on the patient, calcified parasite eggs and foreign body granulomatous reaction were found using histological examination. Due to early detection and surgery, the patient fully recovered with no damage to his eyesight.Although rare, ocular sparganosis should be suspected in a mass of the eye when there is a history of eating frogs and frog poultice applications on eyelids. Early surgical resection is important for a good prognosis.The online version of this article (10.1186/s12886-018-0675-8) contains supplementary material, which is available to authorized users. Sparganosis is a zoonotic parasitic infection caused by migrating plerocercoid tapeworm larvae (sparganum) of the genus Spirometra . Athoughorbit sparganosis in an 8-year-old boy.Because the majority of patients with sparganosis are adults, it is likely to be missed in children . Larvae An 8-year-old boy of Han nationality from the Zhejiang province in China presented to our clinic complaining of having a painless ocular mass in his right eye for 1 month without any medical treatment. Ophthalmological examinations revealed that the anterior orbital tumor was approximately 0.5 × 0.8 cm in size in the right eye. The tumor could be touched and moved under the skin with palpation. Computed tomography (CT) examination showed that the tumor was localized in the intraorbital soft tissue near the medial wall Fig. . Examinaorbit sparganosis was made considering this special lifestyle habit, and the boy was advised by the clinic to have further serological tests. An enzyme-linked immunosorbent assay (ELISA) tested positive for the specific IgG antibody of sparganosis in the serum of the boy. An operation was then performed. After tumor excision, histopathologic examinations showed granulomatous inflammation, necrosis, and basophilic calcified eggs . Tests for acid-fast staining, periodic acid-schiff staining, and periodic acid-silver metheramine staining were negative.The patient reported that he had eaten frogs and applied frog poultice to his eyelids many times since he was 4 years old. The suspicious diagnosis of Based on the presence of calcified eggs and patient history, we confirmed the diagnosis of ocular sparganosis. Visual acuity of the patient remained 20/20 and ocular motility was not restricted after the operation.Li et al. reported that ocular sparganosis accounted for only 12.8% of all cases of sparganosis [Sparganosis is a serious food-borne parasitic zoonosis that is not of high concern due to its rare prevalence. This disease is caused by larvae infection of the Spirometra species. Adult Spirometra cestodes live in the small intestine of carnivores. They release eggs with host faeces to contaminate the environment. The miracidia hatch from the eggs to find a suitable intermediate host. In nature, frogs act as the main secondary intermediate hosts . Humans rganosis . Human organosis . HoweverThe diagnosis of sparganosis in this case was based on imaging, immunologic examination, and pathologic results. Chen et al. reported that cerebral sparganosis presented low and high density lesions on CT images and a ring or a beaded enhancement on enhanced magnetic resonance (MR) or CT images . ImmunolSurgical removal is the most effective for proliferative sparganosis . But conAlthough rare, ocular sparganosis should be suspected in a mass of the eye with a special history. Early detection and treatment is important for a good prognosis.


A 49-year-old woman with a distant history of uterine leiomyosarcoma underwent robotic-assisted laparoscopic partial nephrectomy for a 3.5 cm right renal mass, which was presumed to be a primary renal cell carcinoma. Surgical margins were negative, and the histologic analysis confirmed leiomyosarcoma. Uterine leiomyosarcoma is traditionally a locally aggressive disease with only rare reports of renal involvement. We report a case of a metastatic leiomyosarcoma to the kidney four years following initial treatment for uterine leiomyosarcoma. Uterine leiomyosarcomas (LMS) are rare tumors of the uterus, which comprise roughly 1% of uterine malignancies and one-third of uterine sarcomas . For disA 49-year-old woman presented to the urology clinic with a recently diagnosed 3.5 cm right renal mass found on computed tomography Figure . She wasPathologic analysis by a board certified pathologist revealed negative surgical margins. On low power histologic examination, spindle-shaped cells were seen adjacent to normal renal tubules Figure . ImmunohUterine LMS are aggressive tumors with local recurrence rates ranging from 45% to 73% . MetastaWe have presented a rare case of solitary metastasis to the kidney from a uterine LMS. The patient underwent a transperitoneal robotic-assisted laparoscopic partial nephrectomy, and negative surgical margins were achieved. Thus far, three years after surgery, the patient has remained without evidence of disease. A detailed oncologic history should be obtained upon evaluation of a patient with a new renal mass to identify any potential other primary malignancies that may have caused the renal mass. Consideration should be given for preoperative biopsy for management guidance. In patients with a history of LMS or other aggressive malignancy, wider resection may be considered.


Spontaneous haemorrhage into a parathyroid adenoma is a rare and potentially life-threatening presentation.We report the case of a 45 year old female recently diagnosed with primary hyperparathyroidism who presented with chest discomfort and acute airway compromise due to spontaneous extracapsular haemorrhage into a parathyroid adenoma. Computed tomography (CT) imaging showed a hypopharyngeal haematoma extending 10 cm into the superior mediastinum. Surgical decompression of the cyst followed by enbloc resection of the parathyroid tumour was performed after elective intubation. Calcium and parathyroid hormone (PTH) levels had fallen prior to surgery and remain normal post-operatively.Spontaneous parathyroid haemorrhage should be considered in any patient with unexplained spontaneous cervical haemorrhage, particularly if there is a history of hyperparathyroidism. Initial evaluation of such patients should include serum calcium and PTH as well as imaging. Spontaneous parathyroid haemorrhage is an exceptionally rare but potentially life-threatening presentation. Haemorrhage may be contained within the gland, but often presents as extracapsular haemorrhage extending into the neck or mediastinum, manifesting as acute painful neck swelling, discomfort and cervical ecchymosis. Tracheal compression may lead to airway compromise while compression of the recurrent laryngeal nerve and oesophagus may lead to hoarseness and dysphagia respectively. We describe a case of spontaneous haemorrhage into a parathyroid adenoma presenting with acute airway compromise requiring surgical evacuation.A 45 year old female presented to the emergency department (ED) with a 3 days history of progressive dyspnoea, cough, throat and chest discomfort and palpitations.Eight weeks prior to her ED visit, she was diagnosed with primary hyperparathyroidism  , PTH 367  pg/ml) and 24 h urinary calcium 5.4 mmol/24 h . At her initial assessment she had no symptoms of hypercalcaemia apart from mild constipation. Family history was unremarkable. She received intravenous zolendronic acid and serum calcium fell to a nadir of 2.9 mmol/l. Neck ultrasound reported to show a 6 mm probable parathyroid lesion intimately related to the inferior pole of the right lobe of thyroid. One day prior to her acute presentation, and 6 weeks on from the ultrasound scan, a Sestamibi/SPECT CT revealed a 3.7 cm mass in the right tracheoesophageal groove extending to the posterior upper mediastinum at T1-T2 level , parathyroid hyperplasia or a parathyroid cyst and is thought to occur due to a lack of balance between cell growth and blood supply, somewhat similar to apoplexy seen in other endocrine neoplasia. It may be contained within the parathyroid gland, but often presents as extracapsular haemorrhage extending into the neck and/or mediastinum due to rupture of the haemorrhage through the relatively thin parathyroid tumour wall .Clinical features are determined by the extent of the haemorrhage and degree of hyperparathyroidism. Spontaneous extracapsular parathyroid haemorrhage into the neck may present with acute painful neck swelling, discomfort and cervical ecchymosis . ExternaInitial evaluation of an intracervical haematoma should include serum calcium levels; however patients may be normocalcemic. Imaging with CT, magnetic resonance imagine (MRI) and nuclear medicine is essential to delineate the anatomy of the haemorrhage and, if possible, identify the source of the haemorrhage . In our Surgical exploration is the treatment of choice and offers definitive management of the hyperparathyroidism aswell as addressing local haemorrhage; however timing remains controversial. Airway compromise necessitates emergency surgical exploration and evacuation of the haemorrhage, as in our case. Some suggest a watch-and-wait approach with surgery deferred to 3 months , particuSpontaneous parathyroid haemorrhage is a rare but potentially life-threatening complication. The diagnosis should be considered in any patient presenting with a spontaneous cervical haemorrhage of unknown aetiology, particularly if there is evidence of hypercalcemia, history of hyperparathyroidism or ecchymosis of the neck or chest wall. Initial evaluation should include a calcium and PTH level although these may have fallen if significant glandular haemorrhage has led to infarction of the gland. While some propose conservative management, surgery is the preferred option, particularly in the presence of acute airway compromise.


Fibrovascular polyps (FVPs) are benign tumors that commonly occur in the cervical esophagus. Few cases of FVPs of the hypopharynx have been reported, but there has been no English review concerning hypopharyngeal FVPs (hFVPs). Here, we report a case of a vast hFVP, and we also review 13 hFVP cases found in the English literature (PubMed search) including our case.A 41-year-old man with respiratory distress and regurgitation of a mass was referred to our hospital. Endoscopic and radiographic evaluations were required for diagnosis. The tumor originated from the hypopharynx and covered almost the entire larynx, which caused the airway to narrow. An emergency surgical removal was performed under general anesthesia with orotracheal intubation, and the tumor was completely removed transorally using a laryngeal endoscope. Pathological examination revealed that the mass was a FVP.We investigated patient characteristics, symptoms, polyp size, treatments, and recurrence of 13 hFVP cases. Regurgitation of a mass, dyspnea, and dysphagia were frequently reported symptoms at presentation. All patients were treated by surgical excision with no recurrence. Airway management is critical and tracheotomies were needed in some cases.FVPs are often asymptomatic, and they can suddenly cause respiratory distress from laryngeal blockage. Therefore, we emphasize that when such cases are encountered, airway management and surgical treatment should be considered as early as possible. Fibrovascular polyps (FVPs) are rare benign tumors of the upper digestive tract, and they account for approximately 1 % of all benign tumors in the esophagus and hypopharynx  originating in the esophagus and hypopharynx are FVPs  and surgical approaches  depend on the FVP location, pedicle vascularity, and size. Identification of the polyp’s pedicle is very important for successful removal of the tumor. Small polyps that are less than 2 cm in diameter with thin pedicles may be removed by the transoral approach. Polyps larger than 8 cm in length or those with thick, richly vascularized pedicles should be removed by surgical excision through a cervical or thoracic approach (Kim and Shim Airway management is critical. Tracheotomy or endotracheal intubation allows for controlled ventilation until the polyp can be completely excised. In our review, tracheotomies were performed in 3 patients to protect the airway (Owens et al. We encountered an extremely rare case of a vast hFVP that caused dyspnea, and we also reviewed 13 cases of hFVP. Giant hFVPs are life-threatening because of potential complete airway obstruction and sudden asphyxia due to laryngeal blockage. Thus, when such cases are encountered, airway management and surgical treatment should be considered as early as possible.