Dentinogenic ghost cell tumor – Case report of a rare entity

IntroductionDentinogenic ghost cell tumor (DGCT) is an entity with about 60 cases reported in the literature. It is a benign odontogenic tumor, despite being locally invasive and associated with a risk of local recurrence.Presentation of caseA 47-year-old woman presented with a 2-year-old expansive bone lesion. Radiologically, a multilocular mass was identified in the left superior maxilla, compatible with a tumor of odontogenic origin. She was submitted to an extended resection, and the histology was consistent with a DGCT.DiscussionCentral DGCT affects mainly male patients between the fourth and sixth decades, with a predilection for the posterior portion of the jaws. The symptoms are unspecific, and a vast percentage of patients is asymptomatic. Radiographically a unilocular feature is commonly found, unlike this case. The recommended treatment is extended local resection due to its high recurrence rate.ConclusionDue to its rarity, knowledge of this entity is necessary for a better diagnostic and therapeutic guidance.     

Duodenum-preserving pancreatic head resection in solid pseudopapillary neoplasm—Report of a case

INTRODUCTION

Solid pseudopapillary neoplasm (SPPN) was first characterized by Virginia Frantz in 1959. The duodenum-preserving pancreatic head resection (DPPHR) has been described as treatment for low-grade malignant tumors of the head of the pancreas including eight cases of SPPN.PRESENTATION OF CASE: A 16-year-old white female patient presented with abdominal pain and dyspepsia. Computed tomography scan of abdomen showed a 10 × 9 × 10 cm³ lesion on the pancreatic head. After radiological diagnosis of SPPN the patient was submitted to DPPHR. Resection was achieved with clear margins. Immunohistochemical study demonstrated positivity for progesterone receptor, β-catenin, cytoplasmic paranuclear dot-like CD99, negativity for chromogranin and S100 protein and Ki 67 index of 1%.

DISCUSSION

A large encapsulated pancreatic mass with well-defined borders that contains areas of calcifications and intratumoral hemorrhage on CT scan in a young female is virtually diagnostic of an SPPN. A particular dot-like intracytoplasmic expression of CD99 appears to be highly unique for SPPN

CONCLUSION

DPPHR should be considered in cases of SPPN in the pancreas head if there is no compromise with oncologic radicality.     

Von Hippel–Lindau and myotonic dystrophy of Steinert along with pancreatic neuroendocrine tumor and renal clear cell carcinomal neoplasm: Case report and review of the literature

INTRODUCTIONMyotonic dystrophy of Steinert, DM1, is the most common adult muscular dystrophy and generally is not associated to development on multiple site neoplasm. Von Hippel-Lindau (VHL) disease is a dominantly inherited familial cancer syndrome that is associated to tumors such as hemangioblastoma of the retina or central nervous system, clear-cell renal carcinoma (RCC) and endocrine tumors, most commonly pheochromocytoma and non-secretory pancreatic islet cell cancers. No data exist in literature describing the coexistence of both DM1 and VHL.PRESENTATION OF CASEHerein we report a case of renal and pancreatic neoplasm in a young adult female affected by DM1 and VHL simultaneously.DISCUSSIONDM1 is due to an unstable trinucleotide (CTG) expansion in the 30 antranslated region of the dystrophia myotonica-protein kinase (DMPK) gene, located on chromosome 19q13.3. Several molecular mechanisms thought to be determining the classical DM phenotype have been shown. VHL disease is characterized by marked phenotypic variability and the most common tumors are hemangioblastomas of the retina or central nervous system, clear-cell renal carcinoma (RCC) and endocrine tumors, most commonly pheochromocytoma and non-secretory pancreatic islet cell cancers. The pancreatic manifestations seen in patients with VHL disease are divided into 2 categories: pancreatic neuroendocrine tumor (PNET) as solid tumors, and cystic lesions, including a simple cyst and serous cystadenoma. The surgical approach for these cistic lesions is to consider as golden standard. Blansfield has proposed 3 criteria to predict metastatic disease of PNET in patients with VHL disease: (1) tumor size greater than or equal to 3 cm; (2) presence of a mutation in exon 3; and (3) tumor doubling time less than 500 d. If the patient has none of these criteria the patient could be followed with physical examination and radiological surveillance on a 2/3 years base.⁴ If the patient has 1 criterion, the patient should be followed more closely every 6 months to 1 year. If the patient has 2 or 3 criteria, the patient should be considered for surgery given the high risk of future malignancy. Our patient owned only one criterion but in presence of a second malignant tumor. Our hypothesis for this rare findings is that both DM and VHL might be derived from genetic aberration and these might be linked to a major cancer susceptibility. As far as we know this is the first confirmed case of RCC and neuroendocrine pancreatic cancer occurring concurrently with VHL and, at the same time, DM1. According to this case report and the literature data a VHL should be ruled out in the presence of RCC presenting along with pancreatic cysts/tumor.CONCLUSIONAs far as we know this is the first confirmed case of RCC and neuroendocrine pancreatic cancer occurring concurrently with VHL and, at the same time, DM1. Our hypothesis for the unusual findings is that both DM and VHL derived from genetic aberration and these are linked to a major cancer susceptibility.     

Solid pseudopapillary neoplasm of the pancreas��proposed algorithms for diagnosis and surgical treatment

Purpose  

The incidence of solid pseudopapillary neoplasm (SPN) of the pancreas is rising. Although the evidence for proper management is accumulating, we still lack diagnostic and therapeutic guidelines. In this paper, therefore, we propose an algorithm for diagnosis and treatment of this rare type of tumor.     

Primary tracheal microcystic reticular schwannoma – Case report of a rare neurogenic tumor treated by segmental tracheal resection

Introduction and importanceDiagnosis and treatment of rare diseases are challenging because experience and evidence are limited. Primary tracheal tumors have a low prevalence but awareness of these is important to avoid misdiagnoses. We present a first case of a tracheal microcystic reticular schwannoma in which diagnosis and treatment recommendations were revised several times resulting in months of anxious uncertainty for the patient before complete resection and extensive histopathologic examination were performed.Case presentationA 65-year old woman complained about coughing and cervical pain. Tomographies revealed a tumor of the dorsolateral trachea. After repeated biopsies pathologists and tumorboards in different hospitals could not agree on diagnosis or treatment recommendation, so the impatient patient opted for a complete resection for definite treatment.Clinical discussionNeoplasms of the posterior mediastinum are mainly neurogenic. In rare cases they may originate in the trachea and can be difficult to differentiate from other tumor entities if only biopsies are available. Although the preoperative diagnosis was unclear, tracheal resection was performed successfully. The differential diagnoses of soft tissue sarcoma or myoepithelioma were discarded and a benign, microcystic, reticular schwannoma was confirmed by international reference pathologists.ConclusionMicrocystic, reticular schwannoma is a rare variant and may occur in the trachea. Diagnosis of such rare neoplasms can be difficult, resulting in delayed or suboptimal treatment. Often biopsies are not sufficient to reach a definite diagnosis. But even without preoperative histology, tracheal resection is a safe and feasible option for definitive treatment with very low recurrence rates.     

Peritoneal and nodal recurrence 7?years after the excision of a ruptured solid pseudopapillary neoplasm of the pancreas: report of a case

A solid pseudopapillary neoplasm (SPN) of the pancreas is generally regarded as a neoplasm of low malignant potential and there is rarely recurrence of the disease. A 12-year-old female underwent a pylorus preserving pancreaticoduodenectomy for a ruptured pancreatic SPN following a blunt abdominal trauma. The tumor showed no pathological features suggesting malignant potential. Follow-up imaging studies depicted small nodules adjacent to the superior mesenteric vein 7 years after surgery. A laparotomy was performed, and exploration revealed 3 nodules adjacent to the superior mesenteric vein and 4 small nodules in the mesointestine. All of these lesions were extirpated, and were histologically confirmed to be nodal and peritoneal recurrence of SPN. This case indicates that SPN of the pancreas has a latent ability to recur, regardless of its benign pathological features, and peritoneal spread may be promoted by trauma. A close postoperative follow-up is thus mandatory in all patients with SPN even after a radical resection.     

Management of thymomatous myasthenia gravis – Case report of a rare Covid19 infection sequelae

IntroductionSARS CoV19 infection can predispose to many autoimmune and neurological conditions, thymomatous myasthenia gravis being one of them. The rarity of these case poses therapeutic dilemmas about their further management.Presentation of caseA 61 year old gentleman who had covid19 infection 2 months back, presented with new onset myasthenia gravis and an anterior mediastinal mass. He was diagnosed as a case of anti acetyl choline receptor antibody positive thymomatous myasthenia gravis. The patient was posted for video assisted thoracoscopic excision of thymoma. The procedure was uneventful and patient was discharged with improvement in myasthenic symptoms. Histopathological examination confirmed the diagnosis of WHO Type A Spindle cell thymoma.DiscussionSARS CoV19 infection is associated with an array of autoimmune disorders due to various proposed phenomenon including molecular mimicry and loss of immune tolerance. Post infectious thymomatous myasthenia gravis is extremely uncommon, and can be managed with open, minimally invasive or robotic approach.ConclusionThis is the first documented case of post covid19 infection thymomatous myasthenia gravis to the best of our knowledge, managed with minimally invasive thoracoscopic surgery. Further research is required for documentation of the natural history of the disease and therapeutic outcomes.     

Case report of ovary and fallopian tube as content of a Spigelian hernia – a rare entity

IntroductionSpigelian hernia is a rare entity, comprising 1–2% of all hernias. Various abdominopelvic viscera herniate through the defect in the Spigelian fascia and become incarcerated. Specifically in females, the ovary and/or the fallopian tube can herniate through this defect. This is the third reported case of such a hernia.Presentation of caseWe report here a young lady aged 30 years with right-sided ovarian Spigelian hernia. She presented with a painful lump in the right lower quadrant of abdomen for 2 weeks. On examination, she had a tender irreducible lump below and to the right lateral to the umblilicus. CECT revealed a right-sided ovarian Spigelian hernia. The finding was confirmed at exploration and herniorrhaphy performed. She was discharged on the 3rd postoperative day.DiscussionDiagnosing Spigelian hernia clinically is challenging but radiologic investigations like computed tomography help establish the diagnosis and clarify the contents.ConclusionSpigelian hernia itself is a rare entity and to add to that, herniation of ovary and fallopian tube through Spigelian fascial defect is very rare and a possibility in females.     

Inguinoscrotal extension of a pancreatic collection: a rare complication of pancreatitis—case report and review of the literature

Pancreatitis associated with the extension of a pancreatic collection, pseudocyst or abscess into the groin is a rare phenomenon with few reports in the English literature. Nevertheless, it remains a clinically important differential diagnosis as it may be mistaken for more common pathologies in the groin and with a subsequent unnecessary surgical intervention. A case of this rare complication of pancreatitis is reported, together with a review of the literature.     

Hyperparathyroidism–jaw tumor syndrome: a report of three large kindred

Background  Hyperparathyroidism–jaw tumor syndrome (HPT–JT) is a rare autosomal disease caused by inactivating germ-line mutations of HRPT2 gene, with subsequent loss of Parafibromin expression. It is characterized by familial HPT, ossifying jaw tumors, and other associated neoplasms. Methods  Clinical, histopathological, and genetic features of three large Italian unrelated HPT–JT kindred were assessed. Results  Three different germ-line HRPT2 inactivating mutations were identified. Seventeen affected members and six healthy mutation carriers were found. HPT was diagnosed in virtually all affected patients, at a median age of 36.3 years (range 11–71). In all cases, a single parathyroid involvement was found at surgery, although a metachronous multiglandular involvement causing recurrence after selective parathyroidectomy occurred in 17.6% of cases, after a mean disease-free interval of 13.7 years (range 5–27). Parathyroid carcinoma, atypical parathyroid adenoma, and jaw tumor occurred in one case; uterine involvement in 61.5% of women; other associated neoplasms were thyroid carcinoma (two cases) and renal and colon carcinoma (one case). Immunohistochemistry confirmed the loss of Parafibromin as the distinctive feature of the disease both in parathyroid and uterine tumors. Conclusions  HPT–JT has a frequent single-gland parathyroid involvement and a relatively increased risk of parathyroid carcinoma. The penetrance of the disease is high but incomplete. Regardless of the denomination of the syndrome, jaw tumors occur rarely, while uterine involvement is frequently present. Selective parathyroidectomy may be an effective strategy, but a prolonged follow-up is required because of the risk of recurrences and malignancies. A systematic investigation is also required because of associated malignancies. Best of Endocrine Surgery in Europe 2009     

Diagnosis of a 09 cm pheochromocytoma mistaken to be an intramesenteric pancreatic tumor: Case report complying with the scare guidelines

IntroductionPheochromocytoma is an adrenal medullary tumor of the chromaffin cells first described in 1886, remains an entity not fully discovered that case reports keep showing its diversity in clinical presentation, diagnosis methods treatment and follow up challenges.Case presentationWe report the case of a 47 year old woman with complaining from abdominal pain and major weightless with a 09 cm tumor of the body of the pancreas viewed in imaging with no hormonal secretion but high levels of chromogranine A open surgery conducted that revealed the tumor to be located between the adrenal medulla and the Aorta misleading the team between an adrenal tumor or a zuckerkandl body tumor. The hemodynamic changes after manipulation of the tumor and the histopathology confirming the diagnosis of pheochromocytoma. Our main surprise was the aspect of the tumor bombing in the peritoneal cavity firstly thought to be mesenteric tumor; and the stability of the patient with no premedication until the tumor had been manipulated, enlarging the hypothesis about catecholamine secretion of these tumor.DiscussionThe diagnosis is basically simple when the tumor expresses its catecholamines by biomarkers testing, an imaging. Functional imaging should be used to locate the tumor or its metastasis, the sequence of testing or imaging can vary either it’s a symptomatic tumor or an incidentaloma. Preoperative measures should be taken so no major preoperative complications and the main treatment is the tumor complete resectionConclusionThe malignancy of the pheochromocytoma is no near to be reliably identified which imposes a lifetime follow up of these patients as for metastasis were described even after 40 years after diagnosis.     

Towards a non-operative strategy for severe blunt pancreatic injury—Case report

BackgroundPancreatic injury remains uncommon and the majority occurs in association with injury of other organs. For years, surgery has been advised for those with evidence of pancreatic duct damage. However, a lot of changes were seen in the management of blunt abdominal trauma, with strong support for non-operative management of solid organ injuries. There is strong evidence from paediatric patients that those with severe pancreatic contusion and ductal injury can be managed conservatively.Patients and methodWe present our cases of severe blunt pancreatic injury with ductal damage that were successfully managed non-operatively. We reviewed the literature to find evidence to support this management strategy.ResultOur case report and the literature showed that majority of pancreatic ductal injury have been successfully managed non-operatively without increased morbidity or mortality.ConclusionNon-operative management of blunt pancreatic injury with ductal damage allows the formation of a pseudocyst for delayed drainage safely. This strategy of “induced pseudocyst” is particularly applicable to cases that present late and those with concomitant injuries of other organs. The majority of pseudocysts will subside by themselves. The use of embolization may decrease the need for urgent operation and timely percutaneous drainage may help relieve early symptoms.     

Penile metastasis of osteosarcoma： a rare case report

Dear Editor, Metastasis to the penis is rare, with no more than 500 cases reported to date. Primary tumours of sarcomatous origin are extremely rare, and no case ofosteosarcoma has been described in the literature. Here, we present a case of penile metastasis from osteosarcoma of the ischial tuberosity and discuss its possible metastatic mechanisms and clinical implications.     

Transverse testicular ectopia with scrotal hypospadias but without inguinal hernia – Case report of a rare association

Transverse testicular ectopia is an extremely rare anomaly, in which both the testis migrate towards the same hemiscrotum through the same inguinal canal. It is usually associated with other abnormalities such as persistent Mullerian duct syndrome, true hermaphroditism, inguinal hernia, pseudohermaphroditism, and scrotal anomalies; the association with inguinal hernia being the commonest. We, here, report a case of transverse testicular ectopia in a 12 years old boy having the ectopic testis in contralateral hemiscrotum without any inguinal hernia but having scrotal hypospadias with severe chordee. The diagnosis was made on clinical examination and confirmed on ultrasonography. Trans-septal orchiopexy was done for ectopic testis and hypospadias and chordee were managed by staged repair.     

Case reports - Fracture of a ceramic acetabular insert after ceramic-onceramic THA–a case report

No Abstract available.     

Phytobezoar impaction in a Meckel’s diverticulum; a rare cause of bowel obstruction: Case report and review of literature

IntroductionMeckel’s diverticulum is the most common anomaly of the gastrointestinal tract, occurring in 1–3% of the general population. The most common complication of Meckel’s diverticulum is intestinal obstruction. In this report, we describe a rare cause of intestinal obstruction due to Meckel’s; the phytobezoar. After thorough literature review, the authors found about ten individual reports of the same topic. In all these cases, diagnosis was established intra-operatively.Case presentationA forty-seven-year old male presented to the emergency department with a one-day history of abdominal pain associated with vomiting and constipation. Physical examination diagnostic tests revealed features of intestinal obstruction.DiscussionThe lifetime risk of complications in patients with a Meckel’sdiverticulum is usually small and occurs only in up to 4%. In adults’ intestinal obstruction is the most common complication (40%).ConclusionComplicated Meckel’s diverticulum can have different clinical presentations and can cause bowel obstruction. An association with bezoars impaction is possible and it should be suspected in adult patients presenting with bowel obstruction of unknown causes especially those with high vegetarian diet.     

Extravasation: a rare complication of central venous cannulation? Case report of an imminent erosion of the common carotid artery

Zusammenfassung Eine Extravasation—eine unbeabsichtigte Injektion oder Leckage von Substanzen in das perivaskuläre oder subkutane Gewebe—kann Gewebeschäden verursachen, die je nach Substanz, nach ausgetretener Menge und Konzentration bis hin zur ausgedehnten Weichteilnekrose reichen. Bedeutende Gewebeschäden werden neben Chemotherapeutika u. a. durch Elektrolytkonzentrate, Vasopressoren und hyperosmolare Lösungen verursacht. Um Extravasationen zu vermeiden ist eine Überwachung der Katheterlage, des Infusionsdrucks und der Weichteile um die Einstichstelle erforderlich. Sollte die Prävention versagt haben, muss die Infusion sofort unterbrochen werden. Innerhalb der ersten 24 h sollten die gewebetoxischen Substanzen mit Hilfe von Entlastungsinzisionen und Spülung mit Ringer-Laktat eliminiert werden. Eine später einsetzende Behandlung erfordert ein Débridement. Die Tiefenausdehnung einer möglichen Gewebenekrose wird häufig unterschätzt und sollte deshalb durch MRT beurteilt werden. Wir berichten über eine lebensbedrohliche Halsweichteilnekrose mit drohender Arrosion der A. carotis communis durch Extravasation von Kalium-Clorid-Lösung aus einem dislozierten Jugularis interna Mehrlumenkatheter. Die Pathophysiologie des Schädigungsmechanismus wird diskutiert und es wird ein Überblick über die Inzidenz, das medikamentenabhängige Komplikationsrisiko und die erforderlichen Sofortmaßnahmen bei Extravasationen zentraler und peripherer Venenkatheter gegeben.

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Synovial chondroma in Hoffa’s fat pad: Case report and literature review of a rare disorder

IntroductionSynovial chrondomatosis is a rare disorder characterised by the development of hyaline cartilage from the synovial membrane. Large isolated lesions in the Hoffa's fat pad are an uncommon entity.Presentation of caseA 33 year old gentleman presented complaining of progressive knee pain associated with an enlarging lesion on the anterior aspect of the right knee, with associated locking and giving way. Examination revealed a firm 4 × 5 cm lesion adjacent to the patellar tendon.Subsequent CT and MRI demonstrated a lesion in the inferior aspect of Hoffa’s fat pad, with a second lesion adjacent to the proximal tibiofibular joint, in addition to advanced degenerative changes and a meniscal tear.He proceeded to excisional biopsy. Histological analysis was consistent with a solitary synovial osteochondroma. There were no atypical features suggestive of malignancy.DiscussionSynovial chondromatosis is a rare disorder affecting the synovial joints. The underlying pathophysiology is thought to be metaplastic change of the synovium to hyaline cartilaginous tissue. Transformation to malignancy has been described but is uncommon with an estimated risk of 5%. It is 1.5–2 times as prevalent in males versus females. Symptoms which patients may complain of include pain;locking and giving way; and palpable masses.The management usually entails removal of the mass lesion with or without accompanying synovectomy. Recurrence of disease may occur in up to 15–23% of patients.ConclusionSynovial chrondromatosis is a rare but well recognised condition. Long term follow up is advised in view of the risk of recurrence and malignant transformation.     

Case report: Caesarean scar endometriosis – A rare entity

Introduction and importanceCaesarean scar endometriosis (CSE) is a rare form of endometriosis due to previous surgical scars from obstetrical and gynecological procedures. The incidence of CSE was 0.08% and quite difficult to diagnose.Case presentationA 37-year-old multiparous woman came with intermittent pain in her lower left abdominal region and lump with a bluish color and solid consistency on the left side of the caesarean scar. Ultrasounds findings show a solid mass, measured 45 × 40 × 39 mm with neovascularization color score: 4. In April 2021, we performed mass resection, intraoperatively we found solid mass with no adhesion and infiltration found. Histological examination results confirm external endometriosis from the mass and the fascia was free from endometriosis. The symptoms reported relief after the procedure.Clinical discussionScar endometriosis is largely related to previous abdominal surgery like caesarean section, the mass increase in size during menses and becomes symptomatic. CSE develop ranging 12 months to 21 years and could mimic other hernias or tumor, that's why careful and precise examination is needed. The imaging modality we use in this case was ultrasonography which is the best and most accessible, reliable and cost-effective to diagnose. We performed large surgical excision of the lesion with reconstruction of damaged tissue to prevent recurrence and conversion to malignancy.ConclusionCaesarean scar endometriosis should be considered in women of reproductive age with lower abdominal pain and/or mass at the caesarean scar from previous delivery or following obstetric-gynecologic surgery.