Pancreatic Carcinosarcoma with a Rare Presentation

Introduction/Objective Carcino-sarcoma of the pancreas is a rare tumor and very limited clinical and pathologic data have been reported in the literature. As per World Health Organization (WHO) classification of tumors of the digestive system, the carcinosarcoma of the pancreas is classified together with sarcomatoid carcinoma and anaplastic giant cell carcinomas in undifferentiated carcinoma of pancreas. Carcinosarcoma is a rare entity with very poor prognosis. Methods/Case Report Here we report a rare case of pancreatic carcinosarcoma in a 68 year old male patient who underwent a pancreatoduodenectomy for unilocular cystic mass in the head of the pancreas. The mass grossly replaces the whole head of the pancreas. Histologically, the lesion showed a high-grade spindle cell component and adjacent moderately differentiated adenocarcinoma. On immunohistochemical examination, the carcinomatous component was positive for epithelial markers, and the sarcomatous component was positive for DOG1 and had a focal cytoplasmic staining for S-100. The diagnosis of pancreatic carcinosarcoma was rendered. Treatment options are same as of pancreatic carcinoma. Surgical resection is the best option available for patients. Systemic chemotherapy is indicated for patients with distant metastasis or patients with other contraindications. Despite surgery and adjuvant chemotherapy, recurrence rates are high, and prognosis is poor. However, there are no relevant standard chemotherapies available. Based on the limited number of reported cases, the prognosis of carcinosarcoma of the pancreas appears to be poor. But some cases with long term survival have been reported. There are very few primary pancreatic neoplasms with carcinomatous and sarcomatous components reported in the current literature. Results (if a Case Study enter NA) NA Conclusion This case highlights the importance of familiarity with histopathology of this rare entity, and to order proper immunohistochemical and molecular work-up when there is a suspicious abnormal spindle cell component.

Paratesticular Embryonal Rhabdomyosarcoma with Lung Metastasis – A Rare Case Report

Paratesticular rhabdomyosarcomas (RMS) are very rare malignant tumors arising from the mesenchymal tissues of tunica, epididymis or spermatic cord. They present as painless hard masses in inguinoscrotal region, and large tumors can be mistaken for testicular tumors. They can spread to retroperitoneal lymph nodes or hematogenously metastasize to lung, bones and bone marrow. Here, we report a case of Embryonal RMS with spindle cell component presenting with painless scrotal mass and lung metastasis at initial diagnosis.

Cutaneous Eccrine Adenolipoma with a Spindle Cell Component

Introduction/Objective Cutaneous adenolipoma is a rare, benign variant of solitary lipoma characterized by a mature adipocytic proliferation with entrapped eccrine or apocrine sweat glands. It is found predominately in middle-aged females, with a predilection for lower extremities including the thigh and gluteal regions. Cutaneous adenolipoma is presumed to be a hamartomatous process. Herein, we report a unique case of a cutaneous eccrine adenolipoma with a spindle cell component, namely, an early evolving spindle cell adenolipoma. Methods A 43-year-old female with a recurrent history of hidradenitis presented with a 2.7 cm x 1.5 cm x 0.7 cm soft, mobile nodule on the right posterior thigh. It had been present for at least four months and clinically resembled a lipoma. An excisional punch biopsy was performed for pathological evaluation. A concurrent nevus lipomatosus superficialis was diagnosed from the right perineum. Results Gross examination of the thigh nodule revealed fragments of a tan-yellow, lobulated, fatty lesion. Microscopic examination demonstrated a benign lipomatous proliferation with entrapped eccrine glands associated with a surrounding spindle cell stroma. Initial differential diagnosis included a cutaneous eccrine adenolipoma variant and a cutaneous mixed solitary hamartoma composed of admixed neural, eccrine and lipomatous components. Immunohistochemical staining showed uptake for CD34 and CD10 in the spindle cell stromal component, but negative for desmin and S100. Based on our histopathological findings, our diagnosis of an eccrine cutaneous adenolipoma with a spindle cell component was rendered. This unique lesion displayed features of an evolving spindle cell lipoma with entrapped sweat glands, which may be viewed as an early stage of a dermal spindle cell adenolipoma. Conclusion Cutaneous eccrine adenolipoma is a rare, benign lipomatous neoplasm with entrapped sweat glands that can also show a spindle cell component. We share this rare lesion exemplifying the histomorphological spectrum of a lipomatous hamartoma and to highlight the recognition of a cutaneous adenolipoma.

Thyroid-Like Follicular Carcinoma of the Kidney With Low-Grade Sarcomatoid Component: A Hitherto Undescribed Case

Thyroid-like follicular carcinoma of the kidney (TLFCK) is a rare subtype of renal cell carcinoma, which closely resembles follicular neoplasms of the thyroid and has a distinctive indolent clinical behavior. Until now, a single case of TLFCK with extensive sarcomatoid differentiation has been documented with aggressive clinical course. We present an unusual case of sarcomatoid TLFCK with a low-grade spindle cell component in a 34-year-old male patient, with an indolent course following radical nephrectomy and regional node dissection.

Biphasic Thyroid-Like Low-Grade Nasopharyngeal Papillary Adenocarcinoma with a Prominent Spindle Cell Component: A Case Report

Thyroid-like low-grade nasopharyngeal papillary adenocarcinoma (TLLG-NPPA) is a distinctly rare malignancy of the nasopharynx. Morphologically and immunophenotypically, TLLG-NPPA resembles papillary thyroid carcinoma (PTC) and is characterized by a papillary architecture with PTC-like nuclear features and thyroid transcription factor-1 expression. Recently, some cases of TLLG-NPPA with a spindle cell component have been reported. In this study, we report a very interesting case of biphasic TLLG-NPPA that was predominantly composed of spindle cells, with comprehensive analyses of its clinical, pathological, and immunophenotypical features. A 50-year-old woman presented with a sensation of a foreign body in the nasopharynx. Nasopharyngoscopy and computed tomography demonstrated a pedunculated mass arising from the nasopharyngeal roof. Based on the clinical impression of a nasopharyngeal tumor, an excisional biopsy was performed. At low-power magnification, the nasopharyngeal mass consisted of papillary tumor tissue, the growth pattern and architecture of which resembled those of PTC. The papillae were complex and packed tightly with fibrovascular cores. At high-power magnification, each papillary structure was lined with a pseudostratified cuboidal-to-columnar epithelium. The tumor cell nuclei frequently showed a ground-glass appearance, intranuclear grooves, pseudoinclusions, and membrane thickening and irregularity, resembling the characteristic nuclear morphology of PTC. These histological features were compatible with TLLG-NPPA. Intriguingly, in between the papillary components were spindle cells that appeared very similar to the glandular epithelial cells that imperceptibly merged with the papillary component. This spindle cell component comprised two-thirds of the entire tumor volume. The nuclear morphology of the spindle cell component was similar to that of the papillary component. On immunostaining, both the papillary and spindle cell components were diffusely and strongly positive for thyroid transcription factor-1, cytokeratin 7, cytokeratin 19, vimentin, and Hector Battifora mesothelial-1. In contrast, the tumor cells tested negative for p63, p40, smooth muscle actin, S-100, cytokeratin 5/6, thyroglobulin, BRAF V600E, and Epstein–Barr virus-encoded small RNAs. Only two cases of biphasic TLLG-NPPA exhibiting a prominent spindle cell component had been reported previously in the English literature. When the pathologist receives a primary nasopharyngeal mass with the aforementioned histological features, particularly biopsy specimens with predominant spindle cells, biphasic TLLG-NPPA should be considered in the differential diagnosis. By describing its detailed clinicopathological characteristics, we anticipate that this report will expand the existing knowledge on the spindle cell component associated with TLLG-NPPA.

Expression of TLE1 in Malignant Melanoma With Spindle Cell Morphology: A Potential Diagnostic Pitfall

Objectives. Transducer-like enhancer of split 1 (TLE1) immunohistochemistry is widely used as a biomarker of synovial sarcoma. Spindle cell or desmoplastic melanoma can morphologically mimic synovial sarcoma. The aim of this study was to investigate the expression of TLE1 in melanomas with a spindle cell morphology. Methods. A search of the surgical pathology files resulted in 57 cases of melanomas diagnosed with a spindle cell or desmoplastic component. After review, 8 cases had no definitive dermal spindle cell component and 7 cases had insufficient tissue remaining and were excluded from the study. A total of 42 melanomas were examined for TLE1 immunohistochemistry using a mouse monoclonal antibody (Cell Marque, clone 1F5). Strength and percentage of nuclear TLE1 positivity was graded on a scale from 0 to 3+. Staining for TLE1 was considered positive for 2 to 3+ and negative for 0 to 1+. Results. Nuclear TLE1 expression was identified in 24 (57%) of the 42 melanoma cases with spindle cell morphology (2+, n = 14; 3+, n = 10). TLE1 was considered negative in 18 cases (43%), of which most contained weak staining (1+, n = 14 [33%]) and only a small subset did not show any staining (0, n = 4 [10%]). Conclusion. TLE1 frequently highlights melanomas with spindle cell morphology and is a potential diagnostic pitfall. Therefore, when evaluating spindle cell tumors in which the differential may include both a melanoma and synovial sarcoma, TLE1 expression should be interpreted with caution and in conjunction with an immunohistochemical panel.