Primary mediastinal choriocarcinoma presenting as cutaneous metastasis with resistance to chemotherapy: case report and literature review.

Cutaneous metastases of choriocarcinoma are rare. They may indicate poor prognosis and resistance to chemotherapy. In this report, we present a case of a 25-year-old man who presented with central pleuritic chest pain and right upper arm mass for about a week. The patient also had significant weight loss during the last 5 months along with an episode of generalized seizure. Chest computed tomography scan revealed an 8 cm anterior mediastinal mass. A skin punch biopsy from the right upper arm mass revealed a malignant neoplasm with morphology consistent with metastatic choriocarcinoma. Further work-up revealed multiple lung and brain lesions. Ultrasound of the testes revealed no abnormalities. Several chemotherapy regimens were tried; however, there was no response and the disease showed progression. The patient died 6 months after initial presentation.

A rare case of myxoma presenting as a scrotal lump.

A myxoma is a benign intramuscular gelatinous tumor that is rarely known to arise from the scrotum. It can be confused both clinically and radiologically with other more common scrotal wall lesions such as a sebaceous cyst or an abscess. We report a case of a 54-year-old patient who presented with a scrotal wall swelling that was initially suspected to be an infected cyst or cold abscess on imaging. The final diagnosis of a myxoma was made after surgical excision and histopathological examination.

Primary Sarcomas of the Larynx: A Case Series of Four Different Histopathologic Types.

Primary laryngeal sarcomas are rare. Their nomenclature and classification are similar to soft tissue counterparts; however, there are notable differences between clinical presentation, behavior, treatment, and follow-up. There is sparse information regarding the clinical features, biologic behavior, and treatment modalities of laryngeal sarcomas. To increase our understanding about these tumors, we describe herein an additional series of four cases of different pathologic types of laryngeal sarcomas, including low-grade chondrosarcoma, leiomyosarcoma, well-differentiated liposarcoma, and undifferentiated pleomorphic sarcoma. Our main aim is to upsurge awareness about the morphologic variations of laryngeal sarcomas, to avoid potential pitfalls during histopathologic examination. It is essential to ensure that correct diagnosis, subclassification, and grading are achieved for proper guidance of treatment and clinical follow-up at multidisciplinary team meetings.

Bilateral large cell calcifying Sertoli cell tumours: A testicular preservation approach in a young male.

Testicular Large cell calcifying Sertoli cell tumours (LCCSTs) are extremely rare. The primary challenge in benign LCCSTs, which are typically multifocal and bilateral tumours affecting young males, is to confirm the diagnosis to avoid radical intervention and preserve fertility potential. Patient clinical presentation, laboratory results, diagnostic radiological tests along with confirmatory histopathological studies, are the cornerstones in such cases, nevertheless genetic testing is warranted, as LCCSTs can be part of genetic syndrome such Carney complex. We present a case of bilateral benign LCCSTs in young male managed with testicular preservation approach with characteristic clinical, radiological and histopathological features.

Well-Differentiated Liposarcoma of the Hypopharynx Exhibiting Myxoid Liposarcoma-like Morphology with MDM2 and DDIT3 Co-Amplification.

Well-differentiated liposarcoma (WDL) is one of the most common soft tissue sarcomas in adults. It has a predilection for middle-aged males and arises in deep-seated locations such as retroperitoneum, mediastinum, and spermatic cord. Its occurrence in young individuals at the hypopharyngeal region is an exceedingly rare event. Myxoid liposarcoma (ML)-like changes can seldom occur in some cases of WDL, which makes the diagnosis of WDL more challenging. Amplification of DDIT3 gene in a subset of cases of WDL has shown to be associated with such unique morphology. Herein, we present a case of a 36-year-old gentleman who presented with difficulty in breathing and swallowing for 3 months duration. CT scan of the neck revealed a lesion along the posterior wall of the hypopharynx measuring 3.5 cm. Histopathologic examination revealed a tumor composed of lobules of oval to spindle cells in a prominent myxoid stroma with delicate chicken-wire vasculature. In the vicinity, there were lobules composed of variably sized adipocytes separated by thick fibrous septa that contains atypical hyperchromatic spindle cells. By immunohistochemistry, the tumor cells in both components were immunoreactive for CDK4, but negative for MDM2. Fluorescence in-situ hybridization (FISH) confirmed the presence of MDM2 gene amplification. There was no evidence of FUS-DDIT3 gene rearrangement, however, DDIT3 gene was also amplified. The diagnosis of well-differentiated liposarcoma with prominent myxoid stroma was rendered. This is the first documentation of WDL with ML-like morphology harboring co-amplification of MDM2 and DDIT3 in the hypopharynx.

Extraskeletal myxoid chondrosarcoma of the penis: Description of a rare tumor arising in an unusual location.

Extraskeletal myxoid chondrosarcoma (EMC) is a rare malignant soft tissue tumor that most commonly arises in the extremities. Its occurrence in the genitourinary tract is extremely uncommon. We present an 82-year-old man who was found to have incidental pulmonary metastasis by imaging studies. The biopsy findings from the lung were in favor of metastatic EMC. The primary mass was found to be located at the penile root, which was confirmed to be EMC. This case adds to the few reported cases of EMC arising in the perineal region, and sheds light on the clinical behavior of this soft tissue tumor.

Sclerosing epithelioid fibrosarcoma: in-depth review of a genetically heterogeneous tumor.

First described in 1995 by Meis-Kindbloom et al. as a variant of fibrosarcoma simulating carcinoma, sclerosing epithelioid fibrosarcoma (SEF) is a malignant soft tissue sarcoma characterized by epithelioid cells in dense sclerotic stroma, frequent immunoreactivity for MUC4 and heterogeneous genetic profile with recurrent EWSR1 gene rearrangement. It typically affects middle-age adults with a predilection for the lower extremity. It is believed that SEF is closely related to low-grade fibromyxoid sarcoma (LGFMS), both tumors show overlapping features in morphology, immunophenotype, and molecular profile. In this review, we discuss the clinical, morphologic, and immunohistochemical features of SEF with particular emphasis on its molecular diversity and relation to LGFMS.

Pseudomyogenic hemangioendothelioma of bone with rare WWTR1-FOSB fusion gene: Case report and literature review.

Pseudomyogenic hemangioendothelioma rarely arises in bone. WWTR1-FOSB fusion gene is rarely reported in PMHE of bone. Currently, fusion genes can be used as diagnostic markers in PMHE; however, their prognostic and therapeutic significance is unclear.

Heterotopic Gastric Mucosa in the Ileum: A Rare Cause for Intussusception in Adults.

Intussusception is the leading cause for intestinal obstruction in children. However, it accounts for only 5% of bowel obstructions in adults. Heterotopic gastric mucosa (HGM) can occur anywhere in the gastrointestinal tract; nevertheless, its occurrence in the small intestine is rare unless associated with remnants of vitelline duct (Meckel's diverticulum). Herein, we describe a case of a 33-year-old male who presented with symptoms and signs of intestinal obstruction caused by ileo-colic intussusception, in which polypoid HGM acted as the organic lead point for intussusception. Several cases of intussusception caused by HGM have been reported in pediatric age group; however, this event is exceedingly rare in adults.

A Case of Endocrine Mucin-Producing Sweat Gland Carcinoma: Is it Still an Under-Recognized Entity?

Endocrine mucin-producing sweat gland carcinoma (EMPSGC) is a rare low-grade sweat gland carcinoma characterized by immunoexpression of neuroendocrine markers and mucin production. It occurs most frequently at the head and neck region with strong predilection to the eyelids. Up to 2013, only few cases have been reported. However, in the following years, the number of cases reported has increased significantly, which indicates an upsurge in awareness and increased recognition of this neoplasm. Herein, we describe another case of EMPSGC in a 78-year-old man who presented with a 6-mm skin lesion at the lower eyelid. We discuss the clinical, histopathologic and immunophenotypic features of the tumor with particular emphasis on molecular features and prognosis.

A case of xanthogranulomatous orchitis and its preoperative diagnostic challenges.

Xanthogranulomatous orchitis (XGO) is an extremely rare inflammatory disease of the testis which can mimic testicular tumors. We report a 42-year-old man who presented with left scrotal swelling for one-month duration associated with pus discharge from the overlying scrotal skin. Scrotal ultrasonography revealed an atrophic heterogenous left testis with scrotal wall collection. Surgical scrotal exploration with left simple orchidectomy was performed. By histopathology, the diagnosis of XGO was rendered. The preoperative diagnosis of XGO can very challenging and the diagnosis mainly relies on histopathologic examination. Adequate pathologic sampling is essential to rule out the possibility of co-existing testicular neoplasms.

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Black pleural effusion is a rare entity and may be a diagnostic dilemma. This interactive case discusses the various steps involved to reach the diagnosis. https://bit.ly/3dFJPTS.

Locally Aggressive Giant Cell Tumor of Bone With Pulmonary Distant Metastasis and Extrapulmonary Seeding in Pregnancy.

Giant cell tumor of bone (GCTB) is a locally aggressive benign neoplasm that is associated with a large biological spectrum ranging from latent benign to highly recurrent and occasionally metastatic tumor. In this article, we present a case of a 26-year-old woman who presented with swelling at the left lower ribs during pregnancy. Surgical excision was done, and histopathology showed tumor with features consistent with GCTB. MRI preformed after delivery revealed recurrence of the mass with extensive growth reaching 17 cm with two subcutaneous satellite nodules in the adjacent abdominal wall. positron emission tomography-computed tomography (PET-CT) scan revealed bilateral fluorodeoxyglucose (FDG)-avid lung nodules. Surgical resection was done, and histopathology showed no evidence of malignant transformation. Few months later, the tumor recurred again, with peritoneal deposits. The patient underwent wide massive resection of the recurrent mass and then started on denosumab therapy. Molecular analysis of the tumor detected H3F3A G34W mutation with no copy number alterations. We are presenting this case of GCTB with pulmonary distant metastasis and extrapulmonary seeding to upsurge awareness among clinicians about the possible extreme aggressive biological behavior of GCTB that can mimic the presentation of malignant bone tumor and also to discuss the possible predictive factors of such aggressive behavior.

A case of oncocytic lipoadenoma of the submandibular gland and its diagnostic cytology challenges.

Lipomatous tumors of the salivary gland in general are rare and oncocytic lipoadenomas are even much rarer. Most of the reported cases of oncocytic lipoadenoma were located in the parotid gland and to the best of our knowledge, only four cases reported to arise from the submandibular gland. The preoperative interpretation of the aspirates taken from these lesions can be difficult and challenging. We report a case of a 41 year old gentleman who presented with submandibular gland swelling. CT scan of the neck revealed an oval-shape enhancing mass in the inferior aspect of the right submandibular gland measuring 19 × 12 mm. Fine needle aspiration biopsy (FNAB) showed hypocellular smears with few clusters of epithelial cells having oncocytic appearance. The cytologic findings were interpreted as "oncocytic lesion." Submandibular gland excision was done. Histologically, a final diagnosis of oncocytic lipoadenoma was rendered. We present this rare entity to add to the few cases reported to date and to upsurge awareness during cytologic examination of oncocytic salivary gland lesions.