Axillary nodal metastasis of operated gallbladder carcinoma: remote site of aggression—a case report

Background Gallbladder cancer is a very aggressive type of biliary tract cancer. The only curative treatment is complete surgical excision of the tumour. However, even after surgery, there is still a risk of recurrence of the cancer. Case presentation A 63-year-old gentleman presented with the complaint of a non-healing ulcer at upper abdomen for the last 1 month. He had undergone a laparoscopic cholecystectomy at a private centre 4 months ago. Investigations confirmed the diagnosis of epigastric port site metastasis from a primary from gall bladder adenocarcinoma. After undergoing completion radical cholecystectomy with wide local excision of the epigastric ulcer, he received 6 cycles of concurrent chemoradiotherapy. Eighteen months later, he presented to us with bilateral axillary swellings. Investigations confirmed isolated bilateral axillary metastasis and the patient underwent a bilateral axillary lymphadenectomy (Level 3). However, PET scan after 6 months showed widespread metastasis and the patient succumbed to the illness 1 month later. Conclusion Axillary metastasis probably occurs due to the presence of microscopic systemic metastasis at the time of development of port site metastasis. An R0 resection of the malignancy is the only viable option for effective therapy. The present case highlights the rare involvement of isolated bilateral axillary lymph nodes as a distant metastatic site with no evidence of disease in the locoregional site. However, the prognosis after metastasis remains dismal despite multiple treatment modalities.

Branchial Cleft Anomalies: Embryogenesis, Clinical Features, Diagnosis, and Management

Branchial cleft anomalies are congenital head and neck lesions that arise from incomplete maturation of the branchial apparatus during fetal development. The branchial arches are the precursors of the face, neck and pharynx, and disturbances in embryogenesis can present clinically as cysts, sinus tracts, and fistulas in predictable anatomic locations. However, there remains controversy regarding the precise embryogenesis of branchial cleft anomalies with several competing theories. These lesions often evade diagnosis in early childhood and may present in a delayed fashion after multiple failed interventions. Various diagnostic modalities have been described to facilitate clinical workup. Definitive treatment is complete surgical excision of the lesion. We present a comprehensive review of the literature along with several illustrative cases that highlight the unique challenges of diagnosis and surgical management of branchial cleft anomalies.

Malignant Phyllodes Tumor of the Breast and Pregnancy: A Rare Case Report and Literature Review

Phyllodes Tumor (PT) is a rare fibroepithelial breast tumor that can behave differently depending on its biologic features. Traditionally, PTs are classified by their histologic features into benign, borderline, and malignant. In most cases that were reported, all PTs may recur, but only the borderline and malignant PT can metastasize. PT usually occurs as a breast lump or accidental finding on ultrasound (US) examination. The clinical features include a well-defined breast mass, regular or lobulated. The diagnosis is based on the integration of morphology features, but remains challenging, particularly in the distinction from fibroadenomas. We report a case of a 36-year-old patient who presented for a voluminous breast mass, rapidly growing in the past 3–4 months. At presentation, the patient was 19 weeks pregnant. The breast tumor had the clinical and US aspect of PT. A core needle biopsy was obtained, confirming a benign PT, and local excision was performed with no postoperative complications. The final pathology report showed a borderline PT with close resection margins of 1 mm. Immunohistochemistry (IHC) established the diagnosis of malignant PT with heterologous sarcomatous differentiation. The case was discussed in the multidisciplinary tumor board (MDT) and mastectomy was recommended. The patient fully consented but refused surgery at 25 weeks’ gestation, fearing premature delivery. The right breast was closely monitored by US, and at 9 weeks after the first surgery, signs of local recurrence were detected. At 35 weeks’ gestation, right mastectomy was performed, with no perioperative complications. The pregnancy was closely followed up and no complication were found. The final pathology report describes multiples PT recurrences with heterologous sarcomatous differentiation. The pregnancy outcome was uneventful, and the patient delivered a healthy child vaginally at term with no peripartum complication. Postpartum, a computer tomography (CT) examination of the head, thorax, abdomen and pelvis was performed, with no evidence of metastases. Adjuvant chemotherapy and radiotherapy completed the treatment. The follow-up and CT scan showed no metastases or further recurrence 4 years after diagnosis. In conclusion, diagnosis of PT can be difficult, especially because of the easy confusion with fibroadenoma of the breast. There are rare cases when a pathology exam needs further assessment and IHC is recommended for accurate diagnosis. Although malignant PT is rare and accounts for <1% of all breast cancers, the diagnosis and treatment that are recommended are based on the reported cases. Moreover, when complete surgical excision is achieved, the rates of recurrence and distant metastases are low, and adjuvant therapy might not be necessary.

Acral perineurioma: a case report of a rare neoplasm successfully treated with autologous skin graft reconstructive surgery

Soft tissues perineurioma is a rare nerve sheath tumor that affects most of all the subcutaneous tissue. Even if it could present as a large mass, it is a benign neoplasm for whom a complete surgical excision represents the gold standard treatment. Considering that it usually affects acral sites of young people, can be challenging to perform a reconstructive surgery that allows a full functional recovery. We report the case of a woman in her 20s presenting a perineurioma of the sole of the foot, a nodule of about 2 cm of diameter that compromised the support of the foot on the ground. We performed a radical surgical excision with no recurrence after 3 years of follow up and we obtained a full functional recovery thanks to an autologous full-thickness skin graft.

Radio-pathological correlation of a retroperitoneal teratoma in a 2-year-old girl: a case report

ABSTRACTRetroperitoneal teratoma is a extragonadal germ cell tumor comprising 5% of all teratomas in children, and the third most common retroperitoneal neoplasm in children. This is a case report of a 2 years old girl who presented with abdominal distension and palpable mass. The imaging findings of the mass was characteristic of retroperitoneal teratoma which was confirmed by histopathology report following complete excision. Retroperitoneal tumor is an uncommon tumor in children with characteristic imaging findings. Computed tomography is mainly used to evaluate the extent of the disease. Most of the retroperitoneal tumors are benign and curable with complete surgical excision.

Small but Challenging Conjunctival Melanoma: New Insights, Paradigms and Future Perspectives

Although its incidence has increased over the last decades, conjunctival melanoma (CM) remains a rare but challenging periocular malignancy. While there is currently no recognized standard of care, “no-touch” surgical excision followed by adjuvant treatments is usually recommended. Despite its small size, managing CM is challenging for clinicians. The first challenge is the high risk of tumour local recurrence that occurs in about one third of the patients. The management of locally advanced CM (≥T2) or multiple recurrences may require mutilating surgeries such as orbital exenteration (OE). The second challenge is the metastatic spread of CM that occurs in about one quarter of patients, regardless of whether complete surgical excision is performed or not. This highlights the infiltrative and highly aggressive behaviour of CM. Recently, attention has been directed towards the use of eye-sparing strategies to avoid OE. Initially, wide conservative surgeries followed by customized brachytherapy or radiotherapy have appeared as viable strategies. Nowadays, new biological insights into CM have revealed similarities with cutaneous melanoma. These new findings have allowed clinicians to reconsider the management of locally advanced CM with “medical” eye-sparing treatment as well as the management of metastatic spread. The aim of this review was to summarize the current and future perspectives of treatment for CM based on recent biological findings.

Giant Frontotemporal Cavernous Malformation in a 2-Month-Old Infant: A Case Report and Review of the Literature

<b><i>Introduction:</i></b> Cavernous malformations in the pediatric population are exceedingly rare, especially in infants. Giant cavernous malformations (GCM) are even more rare and have a diameter &#x3e;4 cm. The onset of symptoms predominantly occurs in adulthood, but the rate of hemorrhage is significantly higher in the pediatric population. Similar to non-GCM, GCM can be misidentified as tumors on imaging due to their tumefactive pattern with edema. Here, we present a rare case of a right frontotemporal GCM in a 2-month-old girl, the youngest recorded case in the existing literature. <b><i>Case Presentation:</i></b> A previously healthy 2-month-old girl presented to the emergency department following an increasing frequency of seizure-like activity that began 3 days prior to presentation. Magnetic resonance imaging of the brain with and without contrast characterized a large (5.8 × 4.3 × 4.2 cm) heterogeneous lesion of the right temporal lobe with diffuse scattered blood products of various ages seen throughout the lesion. She underwent a right-sided craniotomy where a gross total excision was achieved. Pathology confirmed the diagnoses of a GCM. The patient’s seizures subsequently resolved, and she continues to do well postoperatively. <b><i>Discussion/Conclusion:</i></b> GCM can be mistaken for tumors due to their large size, cystic nature, and surrounding edema, but a vascular lesion should always remain in the differential diagnosis before operating, even in infants. Surgery is generally recommended in patients that present with a symptomatic hemorrhage, recurrent hemorrhages, persistent seizures despite medical management, or progressively worsening neurological deficits if the GCM is in a safe location. It has been shown that 70–99% of patients undergoing surgery with successful removal of the GCM can achieve seizure freedom 2 years postoperatively. Complete surgical excision of this infant’s GCM was successful in treating her neurologic symptoms; therefore, pathological confirmation of this lesion is critical and should prompt a complete surgical excision.

Desmoplastic fibroma of bone: A morphological and immunohistochemical characterization

Background: Desmoplastic Fibroma (DF) of bone is a locally aggressive and infrequent benign neoplasm. Recently was described a role of vascular endothelial growth factor in the interstitial fibrotic processes. Case presentation: A 13-year-old female presented with pain, swelling and limitation of movements in right forearm. An osteolytic lesion at the distal end of the right radius was shown, with pathologic concentration of Technetium 99 and slight enhancement of soft tissue lesion employing computerized axial tomography. The surgical biopsy showed nodular formations of hyalinized collagen fibers arranged in thick bands with few well-differentiated interstitial fibroblasts / myofibroblasts, focally expressing VEGF-A. Conclusion: The intramedullary neoplastic proliferation is limited by the cortical bone, provoking compression of the intratumorally micro-vessels, favoring both, the extracellular matrix and VEGF-A synthesis. Future research should include therapeutic intervention with anti-CD117 and anti-VEGF-A drugs, with the aim of limiting tumor growth, facilitating the complete surgical excision of the neoplasm. Keywords: desmoplastic fibroma; vascular endothelial growth factor; hyalinization; neoplasm progression.

Cervicothoracic lipoblastoma: three case reports and review of literature

Background Lipoblastoma is a rare benign mesenchymal tumor of embryonal fat affecting mainly children below 3 years of age. It presents usually as a painless rapidly growing soft lobular mass in the extremities, trunk, and less frequently in the head-neck region. Preoperative imaging is used to assess the extent of disease and aid surgical planning. Complete surgical excision without injury to surrounding vital structures is the treatment of choice. Case presentation We report three interesting lipoblastoma cases: mediastinal lipoblastoma with airway compression and ipsilateral diaphragmatic eventration, neck lipoblastoma with intrathoracic extension, and huge thoracic inlet lipoblastoma with compression of common carotid and Lt subclavian arteries, brachial plexus, and ipsilateral diaphragmatic eventration. Complete excision of lipoblastoma mass was done via neck incision in two cases, and the third case required thoracoscopic excision of intrathoracic remnant 6 months later. All three patients had an excellent outcome. Conclusions Management of cervicothoracic lipoblastoma is a surgical challenge due to the potential for rapid growth and extension to different fascial planes; however, successful excision can be achieved via a neck approach. Complete surgical excision is essential to prevent local recurrence and improve the outcome.

Ovarian Germ Cell Tumor with Myasthenia Gravis: Co-incidence or a Possible link?

Teratomas are a common form of germ cell tumor. Teratomas are commonly found in the gonadal organs, such as the ovaries and testes. Treatment of choice for ovarian teratomas is complete surgical excision, which exhibits a good prognosis in benign teratomas; however, chemotherapy treatment is needed for malignant components. Neurological paraneoplastic presentation of gynecological tumors is rare; however, ovarian tumors account for 10% of this presentation. In literature, paraneoplastic limbic encephalitis, anti-N-methyl-D-aspartate receptor encephalitis, and paraneoplastic cerebellar degeneration have been reported in ovarian teratomas and tumors; however, myasthenia gravis has been reported only twice. In both of those cases, manifestation of myasthenia gravis was preceding the diagnosis of ovarian cancer. We describe the first case of a 21-year-old female who presented with new-onset myasthenia gravis after finishing chemotherapy for ovarian teratoma. Another unusual aspect of our case is the rare co-occurrence of gliomatosis peritonei with mature teratoma.