Synovial Sarcoma of the Submaxillary Salivary Gland: A Rare Location and Challenging Diagnosis.

Synovial sarcoma is a high-grade sarcoma. The periarticular region, deep soft tissues, and the extremities are where it is most frequently found. The head and neck regions are rarely affected and salivary gland localization is rather rare, especially the submaxillary gland. The process of diagnosis and therapeutic management remains challenging, particularly in cases with uncommon tumor locations where the establishment of a universal therapeutic consensus is complicated. Early diagnosis and a multidisciplinary approach can lead to success without locoregional recurrence or distant metastases.

Metastatic Epithelioid Hemangioendothelioma of the Bone: A Case Report and Literature Review.

Epithelioid hemangioendothelioma (EHE) is an extremely rare vascular tumor, which can pose a diagnostic dilemma. It affects women more than men and is mainly found in the liver, lung, and bone. To date, there are no known predisposing factors. Limited data are available on the management of EHE at metastatic stages. The only optimal treatments to prevent metastatic dissemination are surgical resection and amputation in addition to radiotherapy at early stages. The oncologist in this rare entity plays an important role in the guided and standardized management of this disease, especially for advanced stages. In this article, we report the case of a 74-year-old patient admitted with swelling on the outer aspect of the right calf associated with pain and total functional impairment of the limb. The diagnosis favored a high-risk vascular tumor resembling EHE, confirmed by bone (tibia) and soft tissue biopsy. The patient underwent staging investigations, revealing diffuse metastases to the liver, bones, and lungs. The objective of this article is to advocate for oncological intervention in this entity, particularly in the advanced stages of the disease. Despite its rarity, the advancement of clinical trials and therapeutic recommendations remains crucial for optimal treatment.

Papillary Neoplasms of the Gallbladder and Extrahepatic Bile Ducts: A Report of Two Cases With Associated Invasive Carcinoma.

Intracholecystic papillary neoplasm (ICPN) of the gallbladder is a macroscopically visible premalignant lesion protruding into the gallbladder lumen, with infrequent association with invasive adenocarcinoma. Intraductal papillary neoplasm of the bile ducts (IPNB) is a non-invasive lesion characterized by intraductal papillary or villous architecture. Both ICPN and IPNB are rare findings in the gallbladder and biliary tract pathology. Diagnosis relies on clinical manifestations, imaging techniques, and comprehensive histological examination. Here, we present two cases: a 63-year-old male with mild abdominal pain found to have a gallbladder mass, diagnosed histologically as ICPN with associated invasive carcinoma; and a 65-year-old female with chronic jaundice and a large tumor mass in the common bile duct, histologically diagnosed as IPNB with associated invasive carcinoma. These cases highlight the importance of a careful and thorough microscopic examination to rule out differential diagnoses and to reveal any potential invasive carcinoma associated with these uncommon lesions.

Gastric Adenocarcinoma With Unusual Clear Tubulopapillary Morphology: A Case Report.

Gastric clear cell adenocarcinoma is an extremely rare variant of papillary adenocarcinoma of the stomach. It is associated with a poor prognosis due to its frequent lymphovascular invasion and also its higher risk of recurrence. It is characterized morphologically by a clear appearance of tumor cells, which can be easily confused with a metastasis of a clear cell carcinoma, particularly of renal origin. Very few cases have been previously reported in the literature, which makes it a very poorly known variant. Here, we report the case of a 64-year-old patient who presented with a polypoid lesion in the pylorus, revealed by epigastric pain and chronic vomiting. Histological analysis showed a gastric clear cell adenocarcinoma with a tubulopapillary architecture. Immunohistochemical examination excluded a metastasis of renal origin. Through this case report, we highlight the importance of recognizing such an uncommon and unusual variant of gastric adenocarcinoma, to prevent any potential misdiagnosis.

Solitary Necrotic Nodule of the Liver: A Benign Mimicker of Malignancy.

Solitary necrotic nodule (SNN) of the liver is an uncommon and benign finding in liver pathology. Typically, it appears as a single and asymptomatic lesion, primarily located at the subcapsular region of the right lobe of the liver. Unfortunately, it is easy to mistake this benign lesion for a primary or secondary neoplastic lesion, making it a potential diagnosis pitfall for liver malignancies. The diagnosis of SNN can be difficult to determine as the imaging findings frequently lack specificity. This brings out the importance of histomorphological examination to accurately identify this lesion, and to rule out any possible malignancies. We report here the case of a 35-year-old woman with a history of squamous cell carcinoma of the cervix, who presented a solitary nodule on her liver that was falsely diagnosed as a metastatic lesion in the liver at imagery. The aim of this article is to highlight the importance of using special stains and immunohistochemical staining for diagnosing SNN and excluding any necrotic metastases of the liver. We demonstrated that the absence of a reticulin meshwork in the necrotic core should prompt consideration of a necrotic metastasis in the liver, rather than a solitary necrotic nodule.

Giant Poroid Hidradenoma of the Forearm.

Poroid hidradenoma represents an uncommon and benign tumor originating from skin adnexa. It falls under the category of sweet duct neoplasms, along with poromas. It affects the elderly population most frequently. Typically, it emerges as a small, distinct, and painless lump beneath the skin's surface, often occurring on the head and neck regions. It is characterized by a low risk of malignant transformation. Accurate identification relies especially on histomorphological analysis considering the intricate resemblance it shares with other tumors originating from eccrine glands. Poroid hidradenoma has only recently been recognized, and only a limited number of cases have been reported in the medical literature. In this instance, we present an unusual occurrence of a giant poroid hidradenoma on the left forearm of an elderly patient.

Pseudoangiomatous stromal hyperplasia: a rare cause of gynecomastia in men.

A 17-year-old male with chest malformation and left breast enlargement underwent surgery for gynecomastia. Histological examination revealed mammary fibrous stroma with ductal hyperplasia and features of pseudoangiomatous stromal hyperplasia. Postoperative follow-up showed no complications, but 8 months later, the patient experienced a mild recurrence with enlargement of the nipple-areolar complex. Although recommended for secondary glandular resection, the patient declined further surgery.

Primary Gastric Signet Ring Cell Carcinoma or Metastatic Lobular Carcinoma With Signet Ring Cells: A Diagnostic Challenge.

Histologically, cells having vacuolated cytoplasm rich in mucin and pushing the nucleus to the periphery are indicative of signet ring cell carcinoma. This condition often affects the digestive system. On the other hand, it is a very uncommon subtype of invasive lobular breast carcinoma, with a higher probability (more than invasive breast carcinoma of no special type {IBC-NST}) to migrate to the stomach, spleen, urinary tract, and uterus. As with other metastatic carcinomas of breast origin, metastatic signet ring cell carcinoma of the breast is often treated with systemic therapies such as chemotherapy or hormonal therapy. However, surgical resection and eventual perioperative chemotherapy are usually recommended in case of primary gastric ring cell carcinomas that are non-metastatic. As a result, misdiagnosis might result in unneeded gastrectomy and chemotherapy, which would result in considerable mortality and morbidity. We report a case of mammary lobular carcinoma with signet ring cells metastatic to the stomach, a variant rarely described and challenging to distinguish from primary gastric signet ring carcinoma.

An Incidental Finding of Endocervical Melanosis in a Polymyomatous Uterus.

Melanocytic lesions, whether benign or malignant, are extremely rare in the cervix and, more particularly, in the endocervical mucosa. Cervical melanosis is a benign entity, most often discovered by incidental findings on a histological study of a surgical specimen resected for another reason. The microscopic examination allows the diagnosis with certainty after ruling out any potential malignancies. The etiopathogenesis remains poorly understood; however, a number of theories have been put forward, such as excessive migration of pigmented cells from the neural crest, trauma, or chronic irritation situations. We report the case of a 40-year-old female patient followed in the gynecology department for a polymyomatous uterus. She underwent a total hysterectomy. The histological and immunohistochemical examinations concluded an incidental finding of cervical melanosis lesions associated with leiomyomas.

Management of an accidental mass in a monorchidic patient discovered during infertility: a case report.

Testicular cancer is the most frequent type of cancer in the young adult men, it is relatively rare. Infertility is an important risk factor for testicular cancer, with a doubled risk of developing cancer compared to the general population. The standard treatment for testicular cancer is the radical orchiectomy, but partial orchiectomy or testicular sparing surgery (TSS) is indicated for small masses, as many experiences, have shown that many small masses found incidentally turn out to be benign. Case presentation: The authors report the case of a patient presented for primary infertility, a clinical examination for left-sided gynecomastia without inflammatory signs. A testicular MRI revealed a 7 mm suspicious nodule in the posterior-inferior aspect of the right testicle, with contrast enhancement in the juxta-tumoral area corresponding to a heterogeneous area on ultrasound. Due to the lesion described on MRI, monorchidism, and azoospermia, a TSS combined with testicular biopsy and testicular sperm extraction was indicated. Clinical discussion: The reference treatment for testicular cancer is the radical orchiectomy, but in some selected situations, partial orchiectomy or TSS is indicated, as many experiences have shown that many small masses found incidentally turn out to be benign. Conclusion: This case suggests that TSS or partial orchiectomy for small nonpalpable testicular masses in monorchidic patients can provide an excellent outcome for the patient.

Extraskeletal myxoid chondrosarcoma: a case report.

Extraskeletal myxoid chondrosarcoma is a rare mesenchymal neoplasm of uncertain differentiation, characterized morphologically by abundant myxoid stroma, a multinodular growth pattern, and uniform cells arranged in strands, clusters, and reticular networks. It usually occurs in adults in the fifth decade, most often in the deep soft tissues of the proximal extremities. The molecular hallmark of this tumor, present in over 90% of cases, is the fusion of NR4A3 with EWSR1 at 22q12.2 or TAF15 at 17q12. Many other tumors with uniform tumor cells embedded in a myxoid matrix can mimic Extraskeletal myxoid chondrosarcoma, and the distinction can be difficult, often requiring immunohistochemistry and/or molecular testing. We herein report the case of an Extraskeletal myxoid chondrosarcoma that occurred in a 74-year-old woman who consulted for a slowly enlarging thigh mass, while highlighting the key morphologic, immunohistochemical, and molecular features of this rare type of soft tissue sarcoma, as well as a summary table gathering diagnostic features of relevance to the differential diagnosis.

Unusual Synchronous Colonic Metastasis of Ovarian Cancer.

Colorectal metastasis is rare and can be confused with primary colorectal cancer. We report the case of a 63-year-old patient who presented with synchronous metastasis of the rectosigmoid junction and ovarian cancer. Initially thought to be a Krukenberg tumor, the diagnosis of metastasis from ovarian origin was confirmed through an immunohistochemical study of the colonic biopsy.

Functional retroperitoneal paraganglioma invading the inferior vena cava in the elderly, a case report and literature review.

INTRODUCTION AND IMPORTANCE: Phaeochromocytomas and paragangliomas are rare neuroendocrine neoplasms that grow outside the adrenal gland and arise from the primitive neural crest cells. The retroperitoneal location is extremely rare with an incidence of 2-8 per million. CASE PRESENTATION: Here we report a case of an 80 years old man presenting with abdominal pain and vomiting associated with hypertensive peaks and weight loss. CT scan showed a retroperitoneal para-aortic tumor invading the inferior vena cava, with significantly elevated urinary catecholamine levels. Histopathological and immunohistochemistry examinations confirmed the diagnosis of paraganglioma. A medical preparation by alpha-blockers was performed. Complete resection of the tumor with the reconstruction of the vena cava was achieved without postoperative complications. After surgery, blood pressure and HbA1c were on the targets and the urinary catecholamine levels were normal. CLINICAL DISCUSSION: The diagnosis of paragangliomas is suspected by clinical symptoms in the case of functional paragangliomas and the confirmation is biological by the plasmatic or urinary catecholamines. Non-functional paragangliomas often represent a diagnostic challenge. In our case, the large size, the location of the tumor, and the invasion of adjacent structures represented a surgical challenge to perform a complete resection. CONCLUSION: In the elderly, this pathology is quite uncommon. Retroperitoneal paraganglioma is a rare location of this type of tumor. Endocrinologists, surgeons, and anesthesiologists should work together to ensure an appropriate diagnosis and treatment of paraganglioma. The gold standard treatment is the complete resection after a medical preparation.

Ovarian plasmacytoma: a case report.

Plasmacytomas are a rare spectrum of plasma cell neoplasms that are single localized tumours, lacking the clinical features of plasma cell myeloma with no radiographical evidence of additional plasma cell tumours. Two clinical variants of plasmacytomas can be distinguished: solitary plasmacytoma of bone and extramedullary (or extraosseous) plasmacytoma. The latter is rare, representing 1% of all plasma cell neoplasms, occurring most frequently in the upper airways. Ovarian localization is exceptional, with only a few cases being reported in the literature. We herein report a case of an ovarian extramedullary plasmacytoma occurring in a 56-year-old woman who consulted for abdominal pain and abdominal mass, while highlighting the main histological and immunohistochemical features of this rare malignancy, along with a thorough review of literature gathering all cases of ovarian plasmacytomas reported to date.

A report of a rare case of paratesticular spindle cell rhabdomyosarcoma in an adult patient.

Paratesticular embryonal rhabdomyosarcoma is a rare malignancy developed from a mesenchymal tissue of spermatic cord, testicular components. Spindle cell rhabdomyosarcoma (SCR) is a variant of embryonal rhabdomyosarcoma affecting the paratesticular region of adult patients and is even rarer. Given the limited guidelines available to manage SCR and the rarity of reported cases, our report aims to discuss a new case of this entity in a 66-years old Moroccan patient. Paratesticular SCR is a very rare tumor and requires attention from urologists to consider this entity as differential diagnosis when suspecting malignancies in the urogenital region.

Endoluminal Leiomyoma of the Gastric Antrum: a Report of a Rare Case.

Endoluminal gastric leiomyoma (GL) of the antrum is a rare benign tumor. In this paper, we report a case of a 72-year-old patient that presented for a 1-month episode of epigastric pain. Imaging found a well-limited tumor in the antrum with extrinsic compression on ulcerated congestive mucosa. A surgical resection based on Finsterer's antrectomy with end-to-side trans-mesocolic gastro-jejunal anastomosis was performed. The histopathological examination in combination with immunohistochemistry diagnosed GL and found a diffuse and marked staining of smooth muscle actin (SMA) and h-caldesmon (h-CD) and negative expression of CD117 and DOG1. The patient was discharged without post-surgical complications and is still alive at the time of this case report writing.

Fibroblastic variant of low-grade endometrial stromal sarcoma mimicking an inflammatory pseudotumor: a case report.

The World Health Organization currently divides endometrial stromal sarcomas into 4 different entities, based on their clinical and pathological features: endometrial stromal nodule, low-grade endometrial stromal sarcomas (LG-ESS), High-grade endometrial stromal sarcomas, and undifferentiated uterine sarcoma. The fibroblastic variant of LG-ESS is rare and is usually made of small tumoral cells of oval to fusiform shape, demonstrating low cytologic atypia and low mitotic activity, which can lead to confusion with a benign myofibroblastic proliferation. We hereby report a rare case of a fibroblastic variant of LG-ESS in a 37-year-old woman presenting abundant metrorrhagia, which was initially misdiagnosed as an inflammatory pseudotumor before proofreading in our laboratory, along with a review of a histological and immunohistochemical findings, aiming to help pathologists avoid this diagnosis pitfall.

Xeroderma pigmentosum and rhabdoid tumor of the kidney: A very rare case report association.

The aim of this observation was to report an exceptional association of xeroderma pigmentosum and rhabdoid renal tumor in a 7-year-old girl, diagnosed with imaging and treated by adjuvant chemotherapy.

Biliary hamartoma: a report of two cases.

Biliary hamartoma, also known as biliary micro hamartoma or Von Meyenburg complex, is a rare benign liver lesion, thought to be a ductal plate malformation rather than a true neoplasm. It is often seen incidentally on imagery or surgery as multiple small subcapsular nodules, scattered throughout the liver, making it likely to be mistaken for metastatic nodules. The histological presentation can also be deceptive, leading to the misdiagnosis of an adenocarcinoma of hepato-biliary differentiation or a metastasis. We hereby present two cases of biliary hamartoma, found incidentally on imagery and surgery, the first one in a 94-year-old woman, and the second in a 48-year-old man, which was initially misdiagnosed as an adenocarcinoma, along with a discussion of key clinical and pathological findings to help avoid this diagnostic pitfall.

Insular thyroid carcinoma in a young Moroccan man: Case report and review of the literature.

Introduction: Insular thyroid carcinoma (ITC) was defined as a rare malignant thyroid cancer standing in an intermediate position between the well-differentiated (papillary and follicular) and the anaplastic thyroid carcinomas. The incidence was estimated around <1% and 10% worldwide. Despite its rarity, it remains the main cause of death from non-anaplastic follicular cell-derived thyroid cancers. Case presentation: A 27-year-old single male admitted for a history of a thyroid nodule and intrathoracic extension; with local mass effect, deviating the brachiocephalic trunk to the right. He underwent a total thyroidectomy. Histopathological examination showed a poorly differentiated insular thyroid carcinoma. Radioactive iodine-131 therapy was administred at a dose of 100 mCi, and the patient was maintained on TSH-suppressive therapy. Ultrasensitive Thyroglobulin measurement after thyroxine withdrawal, taken 2 years after radioactive iodine treatment was undetectable as well as thyroid antithyroglobulin antibodies. Conclusion: Our clinical case would enrich the global registry of insular thyroid carcinomas' cases. The main challenge is early detection, aggressive intervention, and close follow-up of affected patients. The advancement in ultra-deep sequencing technologies, will contribute in the development of novel targeted therapies aiming to reduce morbidity and mortality and improve the outcomes in PDTC patients as well.