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A Possible Distinct Molecular Subtype (Quintuple-Wildtype) of Metastatic Colorectal Cancer in First-Line Anti-EGFR Therapy with Cetuximab Plus FOLFIRI - Palliative Precision Therapy and a Multidisciplinary Treatment Approach: Interim Analysis of the IVOPAK II Trial with Early Results.
Wein, A, Stoehr, R, Kersting, S, Siebler, J, Merkel, S, Busse, D, Wolff, K, Ostermeier, N, Neufert, C, Vitali, F, et al
Oncology. 2022;(1):1-11
Abstract
OBJECTIVE The study aimed to prospectively evaluate a new molecular biomarker panel (KRAS, NRAS, BRAF, PIK3CA, and ERBB2) for palliative first-line treatment of colorectal cancer (CRC), including a multidisciplinary treatment approach. The rate of secondary metastasis resections was assessed. PATIENTS AND METHODS A total of 40 patients with definitively nonresectable metastatic CRC were enrolled from 10 centers before the interim analysis (June 2019) of the IVOPAK II trial (Interdisciplinary Care with Quality Control in Palliative Treatment of Colorectal Cancer). After determination of 5 molecular biomarkers in the tumor (KRAS, exons 2-4; NRAS, exons 2-4; BRAF V600E; PIK3CA; and ERBB2), patients in the IVOPAK II study received FOLFIRI plus cetuximab for all-RAS/quintuple-wildtype disease and FOLFIRI plus bevacizumab in the case of RAS mutations. The current article presents the early description of the clinical outcome of the interim analysis of IVOPAK II comparing the all-RAS/quintuple-wildtype and RAS-mutations populations, including a multidisciplinary-treated case report of a quintuple-wildtype patient. RESULTS The quintuple-wildtype population treated with FOLFIRI plus cetuximab in first-line exhibited a significantly higher response rate and enhanced early tumor shrinkage in the interim analysis than the RAS-mutations population, as well as a high rate of secondary metastatic resections. CONCLUSION Initial results of this new biomarker panel (quintuple-wildtype) are promising for anti-EGFR therapy with cetuximab plus doublet chemotherapy (FOLFIRI) in first-line treatment of metastatic CRC. These results warrant confirmation with higher case numbers in the IVOPAK II trial.
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2.
Clear Cell Variant of Solid Pseudopapillary Neoplasm of the Pancreas: A Report of a Rare Variant and Review of the Literature.
Costales, C, Mehta, A, Kulkarni, S, Larson, BK
International journal of surgical pathology. 2019;(5):535-540
Abstract
The clear cell variant of solid pseudopapillary neoplasm (ccSPN) of the pancreas was first described in 2006. In this article, we report a case of this rare variant and review the few published reports. Both the current and previous reports show that ccSPN has several morphologic differences from conventional SPN, including clear vacuoles, fewer pseudopapillary formations, more solid/diffuse architecture, less hemorrhage, and fewer cholesterol clefts. Some of these features peculiar to ccSPN, such as solid/diffuse architecture, have been proposed to suggest aggressive behavior, though reports of ccSPN are rare and often have limited clinical follow-up. ccSPN also appears to occur more frequently in males than conventional SPNs. These clinical and pathologic features lead to unique set of differential diagnostic considerations for ccSPN, including metastatic renal cell carcinoma, perivascular epithelial cell tumor, and clear cell variants of other carcinomas. These unique features, atypical differential, and uncertain prognostic ramifications all make ccSPN an important variant to be aware of and report.
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Humoral Hypercalcemia in Uterine Cancers: A Case Report and Literature Review.
Motilal Nehru, V, Garcia, G, Ding, J, Kong, F, Dai, Q
The American journal of case reports. 2017;:22-25
Abstract
BACKGROUND Paraneoplastic hypercalcemia is a well-described complication associated with a variety of malignancies. However, its incidence in gynecological malignancies is low. CASE REPORT A 53-year-old woman presented with progressive abdominal distention and irregular vaginal bleeding of several weeks' duration. A contrast CT abdomen and pelvis was significant for a mass in the lower uterine/cervical region, multiple peritoneal and omental masses, enlarged pelvic and paraaortic lymph nodes, and large-volume ascites. A pelvic exam revealed a fungating vaginal mass, with biopsy showing a high-grade tumor with immunohistochemical staining positive for vimentin, CD10, and cyclin D1, consistent with endometrial stromal sarcoma. During her hospitalization, the patient became increasingly lethargic. Workup showed severe hypercalcemia and evidence of acute kidney injury. The patient did not have evidence of bony metastatic disease on imaging studies. Further laboratory evaluation revealed an elevated PTHrP of 301 pg/mL (nl 14-27), a depressed PTH level of 3 pg/mL (nl 15-65), and a depressed 25-OH vitamin D level of 16 ng/mL (nl 30-100), consistent with humoral hypercalcemia of malignancy. The patient was treated with pamidronate, calcitonin, and intravenous fluids. She eventually required temporary hemodialysis and denosumab for refractory hypercalcemia, which improved her electrolyte abnormalities and clinical status. CONCLUSIONS Uterine malignancies of various histologies are increasingly recognized as a cause of humoral hypercalcemia. They are an important differential diagnosis in a woman with hypercalcemia and abnormal vaginal bleeding or abdominal symptoms.
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Unusual focal keratin expression in plexiform angiomyxoid myofibroblastic tumor: A case report and review of the literature.
Quero, G, Musarra, T, Carrato, A, Fici, M, Martini, M, Dei Tos, AP, Alfieri, S, Ricci, R
Medicine. 2016;(28):e4207
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Abstract
BACKGROUND Plexiform angiomyxoid myofibroblastic tumor (PAMT), also known as plexiform fibromyxoma, is a rare distinctive benign intramural tumor, typical of gastric antrum, commonly causing mucosal ulceration with upper gastrointestinal bleeding and anemia, effectively treated by complete surgical resection usually accomplished by distal gastrectomy. METHODS AND RESULTS We herein report a 47-year-old man presenting with a syncopal episode, regurgitation and epigastric discomfort, bearing a gastric antral myxoid plexiform tumor positive for α-smooth muscle actin, vimentin and, partially, for caldesmon, desmin, and CD10; CD117, DOG1, CD34, S100, CAM5.2, CK20, CK7, EMA, p53, CDX2, chromogranin A, synaptophysin, anaplastic lymphoma kinase, Melan-A, and HMB-45 were all negative. All these features are typical of PAMT. Of note, focal positivity for AE1/AE3 and pan-CK KL1 was also present. CONCLUSIONS The finding of a focal keratin expression in PAMT contributes to enlarge the immunophenotypic spectrum of this tumor type and is relevant for avoiding presurgical misdiagnoses which could ultimately lead to inappropriate overtreatment of patients with PAMT.
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HMB-45 negative angiomyolipoma of the orbit: a case report and review of the literature.
Lin, CY, Tsai, CC, Kau, HC, Yu, WK, Kao, SC, Liu, CJ
BMC ophthalmology. 2016;:8
Abstract
BACKGROUND Angiomyolipoma is a benign mesenchymal tumor composed of variable amounts of smooth muscle, adipose tissue and thick-walled blood vessels, and usually named PEComas (perivascular epithelioid cell tumors). PEComas share overlapping histopathological features with epithelioid cells along a perivascular distribution and characteristic immunohistochemistry with coexpression of myoid and melanocytic markers (HMB-45 /or Melan-A). We report the first case of primary orbital angiomyolipoma with negative melanocytic marker. CASE PRESENTATION An 80-year-old Asian woman had a 2-year history of progressive swelling in the left upper eyelid. External examination revealed 3 cm of relative proptosis of the left eye and a palpable mass in the left superonasal orbit. Computed tomographic scan demonstrated a circumscribed, heterogeneous orbital mass. Excision biopsy was done and the histological finding demonstrated the orbital mass was composed of mature adipocytes, intermingled with spindle or oval-shaped cells, and accompanied by thick-walled blood vessels. Immunohistochemically, tumor cells were positive for CD34 and HHF-35, but negative for cytokeratin, HMB-45 and Melan-A. The diagnosis of angiomyolipoma was made. No recurrence was noted at 2-year follow-up. CONCLUSION In our case, the HMB-45 negativity may be explained by the rarity of the epithelioid cells, and the HMB-45 positivity is often weaker or absent in spindle cells. Angiomyolipoma, although rare, should be added to the differential diagnosis of space-occupying orbital lesion.
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Primary mucosal malignant melanoma of the cervix: case report and review of the literature.
Cetinkaya, K, Benzer, E, Dervisoglu, H
Tumori. 2015;(5):e147-50
Abstract
The incidence of primary mucosal malignant melanoma (PMMM) is 1.3% among all malignant melanomas (MM). Cervical involvement is very rare; the number of cases of cervical PMMM reported so far is around 80. In our patient, a dark color, 2-cm diameter, nonulcerated tumor formation was observed upon examination of the cervix. Tumoral tissue consisted of atypical melanocytic cells containing numerous mitotic figures. In immunochemical studies, S-100, Melan-A, and HMB-45 positivity were observed. The tumor was 20 mm in invasion depth, Breslow IV, and FIGO stage IB1. Radical surgery was followed by adjuvant radiotherapy, and subsequently interferon treatment was applied. Examination and scans 20 months after surgery were free from tumor.
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Primary malignant myopericytoma of the left atrium--a tumor of aggressive biological behavior: report of the first case and review of literature.
Mainville, GN, Satoskar, AA, Iwenofu, OH
Applied immunohistochemistry & molecular morphology : AIMM. 2015;(6):464-9
Abstract
We report a case of malignant myopericytoma arising in the left atrium with brain, skeletal, and liver metastases, which, to our knowledge, is the first report of this rare entity in this anatomic location. A 52-year-old man presented with progressive blackening of his left field of vision. Magnetic resonance imaging and a computed tomography scan of the brain and thorax showed a heterogeneous mass in the right occipital lobe and a large left atrial floor mass. Excision of the atrial mass showed a circumscribed but unencapsulated malignant spindled neoplasm with a perivascular concentric cellular arrangement punctuated by sheets of tumor necrosis. The cells were round to spindled with eosinophilic cytoplasm and indistinct borders. Focally, the tumor infiltrated cardiac muscle. By immunohistochemistry, the cells were positive for smooth muscle actin and negative for desmin, H-Caldesmon, S-100, HMB-45, and Melan-A. The features were prototypic for malignant myopericytoma. Eight months after initial presentation, the patient is alive with metastatic disease.
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Unusual presentation of melanoma of unknown primary origin: A case report and review of literature.
Bankar, S, Patkar, S, Desai, S, Shrikhande, SV
Journal of cancer research and therapeutics. 2015;(4):1025
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Abstract
Malignant melanomas often present with metastasis to regional lymph nodes. Patients are also known to present with metastatic nodes in the absence of a known primary. However metastatic melanoma involving the stomach is rare in the scenario of an unknown primary lesion. We present a case of a 41-year-old lady who was treated for nodal disease in the right axilla 8. months earlier with an unknown primary. She later presented to us with a recurrent right axillary swelling and on evaluation was found to also have a nodular deposit on the greater curvature of the stomach. Histopathology of the deposit in conjunction with immunohistochemical markers S.100P, Melan-A and HMB-45, confirmed the diagnosis of malignant melanoma. The patient underwent surgical excision of both lesions and is now on regular follow-up.
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Bevacizumab in metastatic colorectal cancer and carcino-embryonic antigen kinetics: case report and review of literature.
de Andrade, DP, Lima, JP, Lima, AD, Sasse, AD, dos Santos, LV
Anti-cancer drugs. 2011;:S15-7
Abstract
Bevacizumab-containing treatment is one of the approved first-line options for the management of metastatic colorectal cancer (CRC). Carcino-embryonic antigen (CEA) is a reasonable tumour marker for monitoring the efficacy of treatment. We report here a case of a 58-year-old male patient with metastatic CRC. He received first-line chemotherapy consisting of the mFOLFOX6 regimen plus bevacizumab. Initially he had an increase in CEA after which the level reduced, suggesting the occurrence of a CEA flare. The patient experienced a good response to therapy. In recent literature, CEA flare was identified as a good predictor of response to first-line chemotherapy.
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Calponin expression in laryngeal myoepithelial carcinoma and its prognostic implications: a case report and literature review.
Mao, YJ, Luo, XM, Zhou, SH, Zheng, ZJ
The Journal of international medical research. 2010;(2):711-9
Abstract
A case report of laryngeal myoepithelial carcinoma (MEC) is presented and the literature concerning prognostic factors in MEC is reviewed. A 61-year old man was admitted to hospital with hoarseness and progressive dyspnoea. On examination, both vocal cords were fixed in the midline with a glottic fissure of only 3 mm. No tumour was seen, but the subglottis was not completely visible. A computed tomography scan showed a soft mass below the right vocal cord obstructing two-thirds of the larynx. On suspension laryngoscopy, a dull mass (1.5 x 1.5 cm) was seen below the right vocal cord, which was malignant on frozen biopsy. A total laryngectomy was performed and the patient received radiotherapy. He died of recurrence 25 months later. The tumour was positive for cytokeratin 14, S-100 protein and calponin. MEC of the larynx is extremely rare. The clinical behaviour of MEC is variable and prognostic factors have been poorly analysed. Calponin expression may be a prognostic factor, but other factors also affect the outcome in MEC.