Osteoclast-Like Giant Cell Tumors of the Pancreas: Clinical Characteristics, Genetic Testing, and Treatment Modalities.

OBJECTIVES: This study sought to better characterize patient characteristics, treatment options, and outcomes for osteoclast-like giant cell carcinoma of the pancreas, a rare subtype of pancreatic adenocarcinoma. METHODS: This is a retrospective study of all patients with osteoclast-like giant cell carcinoma of pancreatic origin treated at Mayo Clinic from 2000 to present. Baseline patient characteristics, treatment modalities utilized, and outcomes were compiled. Overall survival (OS) and progression-free survival were assessed using Kaplan-Meier analysis with a significance level of P ≤ 0.05. RESULTS: Fifteen patients met criteria for inclusion. Four patients had distant metastases at diagnosis, the remaining 11 with locoregional disease. Median OS for the entire cohort was 11 months. Metastatic disease was associated with significantly shorter OS (3.5 vs 14.1 months; P = 0.005). Three patients had no evidence of disease at time of analysis; all 3 were treated with complete resection followed by adjuvant chemotherapy. CONCLUSIONS: Osteoclast-like giant cell carcinoma of the pancreas is an aggressive malignancy with poor prognosis. For patients with locoregional disease, surgical resection followed by adjuvant chemoradiation may play a role in extended disease-free survival. Metastatic disease presents a challenging entity to treat with little data to support any effective chemotherapy regimens.

Pleomorphic giant cell carcinoma of prostate: Rare tumor with unique clinicopathological, immunohistochemical, and molecular features.

Pleomorphic giant cell carcinoma (PGCC) of the prostate is a rare entity categorized as a variant of prostatic acinar adenocarcinoma in the 2016 World Health Organization (WHO) classification system. PGCC differs from conventional prostatic adenocarcinoma by having bizarre, markedly enlarged, and pleomorphic cells. It differs from high grade urothelial carcinoma by negativity for urothelial differentiation markers, and can be distinguished from sarcomatoid carcinoma by lack of spindle cells. Including two new cases described herein, there have been 51 cases of prostate PGCC reported in the English literature. Clinical features shared by cases of prostate PGCC include poor prognosis, occurrence in older patients, and frequent association with prior therapy. Pathologic features common to cases of prostate PGCC include admixture with a high-grade conventional prostate carcinoma component and absent or reduced expression of prostate differentiation markers. More recent studies have begun to elucidate the molecular characteristics of PGCC, detecting specific mutations and chromosomal translocations, and showing evidence of a high degree of molecular instability in these tumors. We report novel findings in two cases of PGCC including a PIK3CA p.His1047Arg mutation not previously described. One of our cases is the first to clearly demonstrate chronological loss of prostate markers during dedifferentiation from prior conventional prostate carcinoma to PGCC. Herein, we present our two new cases and comprehensively review the literature on all reported cases of PGCC with critical commentary on findings in cases of this rare tumor.

The diagnostic utility of zinc E-box 1 (ZEB1) transcription factor for identification of pulmonary sarcomatoid carcinoma in cytologic and surgical specimens.

OBJECTIVE: Although uncommon, pulmonary sarcomatoid carcinoma carries a worse prognosis due to poor chemotherapeutic response. Currently, a histologic spindle and/or giant cell component indicates sarcomatoid differentiation, with zinc E-box binding homeobox 1 (ZEB1) implicated in promoting epithelial-mesenchymal transition. However, diagnostic use of ZEB1 in limited specimens, including cell block (CB) preparations, remains unclear. MATERIALS AND METHODS: Pulmonary sarcomatoid (SARC, n = 15), typical (TC, n = 10) and atypical carcinoid (AC, n = 10), small cell (SCLC, n = 8) and large cell neuroendocrine carcinoma (LCNEC, n = 9), squamous cell carcinoma (SQ, n = 7), and adenocarcinoma (ADC, n = 7) CBs along with 69 SARCs, 20 TCs, 21 ACs, 9 SCLCs, 10 LCNECs, 71 SQs, 402 ADCs, 16 large cell carcinoma (LCC) and 17 other thoracic tumor (OT) surgical specimens between 2007 and 2018 were retrieved. ZEB1 (Sigma Aldrich, St. Louis, Mo and Novus Biological, Centennial, Colo) immunohistochemistry was graded 1+ to 3+, with ≥1+ and >5% staining considered positive. RESULTS: Nuclear ZEB1 was seen in 80% SARC (12/15), 0% TC (0/10), 0% AC (0/10), 12.5% SCLC (1/8) and 11.1% LCNEC (1/9), 0% SQ (0/7), and 0% ADC (0/7) CBs. In surgical specimens, 75.4% SARCs (52/69), 0% TCs (0/20), 0% ACs (0/21), 11.1% SCLCs (1/9), 30% LCNECs (3/10), 0% SQs (0/71), 0.2% ADCs (1/402), 12.5% LCCs (2/16), and 11.8% OTs (2/17) demonstrated ZEB1. ZEB1 sensitivity and specificity in cytology and surgical specimens were 80% and 96.1%, and 75.4% and 98.1%, respectively. CONCLUSIONS: ZEB1 is sensitive and highly specific for pulmonary sarcomatoid carcinoma in limited cytologic and surgical specimens. Diagnostic pitfalls include high-grade neuroendocrine tumors and large cell carcinoma, which are resolvable by morphologic considerations.

Gestational pulmonary giant cell carcinoma - An autopsy report.

Cancer is an uncommon event in pregnancy. The most common gestational cancers arise from the breast and cervix. Although lung cancer is the most common malignancy, its occurrence in pregnancy is a distinctly rare event. Diagnosing it during pregnancy is an additional challenge as the pregnancy, common respiratory ailments, and the lung cancer can have overlapping symptoms. We report an uncommon histological variant of lung cancer - giant cell carcinoma, which resulted in a fatal outcome in a 26-year-old pregnant woman. A high level of suspicion and vigilance should be exercised during pregnancy to diagnose such rarer entities.

Osteoclast-like Giant Cell-type Pancreatic Anaplastic Carcinoma Presenting with a Duodenal Polypoid Lesion.

Osteoclast-like giant cell-type (OCGC) anaplastic carcinoma is a rare variant of pancreatic ductal adenocarcinoma, and its imaging characteristics and progression pattern have not been fully clarified. The patient was a 73-year-old man who had been incidentally found to have a pancreatic head tumor. Computed tomography demonstrated a 3-cm marginally enhanced mass at the pancreatic head, continuing toward the duodenum. Diffusion-weighted magnetic resonance imaging showed a retained diffusion capacity. Duodenoscopy revealed a 1.5-cm polypoid lesion, covered by a dirty coat, near the major papilla. Surgical material revealed OCGC pancreatic anaplastic carcinoma protruding to the duodenum, accompanied by multiple hemorrhagic foci and hemosiderin precipitations.

Giant cell carcinoma of the lung presenting as an isolated cyst containing air: A case report.

INTRODUCTION: Pulmonary sarcomatoid carcinomas (PSCs) are rare tumors within the sarcomatoid carcinoma group. Giant cell carcinoma of the lung (GCCL) is a rare type of PSCs that consists entirely of highly pleomorphic tumor giant cells; the prognosis is poor. PATIENT CONCERNS: A patient presented with a single cyst and was diagnosed with GCCL. The patient was a 59-year-old male who was admitted to the hospital with a cough. A chest computerized tomography (CT) scan showed a single, thin-walled cyst containing air in the left upper lobe of the lung. Bronchoscopy revealed chronic bronchitis. The initial diagnosis was pulmonary infection and the patient was treated with antibiotics. The cyst wall increased in thickness, and the cyst eventually formed a cavity. DIAGNOSIS: Surgery was performed, and a diagnosis of GCCL was established. The stage was pT1bN1M0 (equal to stage IIB). INTERVENTIONS: The patient underwent video-assisted thoracoscopic surgery and 4 cycles of adjuvant chemotherapy consisting of cisplatin and docetaxel. After 9 months, the patient occurred mediastinal lymph node metastasis, and received radiotherapy (60Gy/30F). OUTCOMES: His prognosis was good without progression (complete response) based on serial CT scans over 9 months of follow-up evaluations, then the patient occurred mediastinal lymph node metastasis. The patient lived during 30 months of follow-up, after which he was lost to follow-up. CONCLUSION: A solitary pulmonary parenchymal cystic lesion usually suggests an infectious disease or congenital abnormality; however, a cystic lesion is occasionally encountered in GCCL.

c-MET Overexpression as a Poor Predictor of MET Amplifications or Exon 14 Mutations in Lung Sarcomatoid Carcinomas.

INTRODUCTION: MNNG HOS transforming gene (MET) abnormalities such as amplification and exon 14 mutations may be responsive to targeted therapies. They are prevalent in lung sarcomatoid carcinomas (LSCs) and must be diagnosed as efficiently as possible. Hypothetically, c-MET overexpression by immunohistochemistry (IHC) may prove effective as a screening test for MET abnormalities. METHODS: Tissue samples were obtained from consecutive patients with a resected LSC in four oncologic centers. IHC was performed using the SP44 antibody (Ventana, Tucson, Arizona) and evaluated using the MetMab score and H-score. Fluorescence in situ hybridization was applied with the dual color probe set from Zytovision (Clinisciences, Nanterre, France). True MET amplification was diagnosed when MET gene copy number was 5 or greater and the ratio between MET gene copy number and chromosome 7 number was greater than 2. All MET exon 14 alterations including those affecting splice sites occurring within splice donor and acceptor sites were detected in the routine molecular testing on genetic platforms. RESULTS: A total of 81 LSCs were included. Fourteen (17%) exhibited positive IHC using the MetMab score and 15 (18.5%) using the H-score. MET amplification was detected in six tumors (8.5%) and MET exon 14 mutation in five (6%). A weak positive correlation between IHC and fluorescence in situ hybridization was found (r = 0.27, p = 0.0001). IHC sensitivity for MET amplification was 50%, with a specificity of 83%, positive predictive value of 21.4%, and negative predictive value of 94.7%. IHC sensitivity for MET exon 14 mutations was 20%, with a specificity of 83%, positive predictive value of 7%, and negative predictive value of 94%. CONCLUSION: IHC is not a relevant screening tool for MET abnormalities in LSC.

Histomolecular profiling of pleomorphic, spindle cell, and giant cell carcinoma of the lung for targeted therapies.

In pleomorphic, spindle cell, and giant cell carcinoma (PSCGC) of the lung, we wondered if an integrated diagnosis including morphological and immunohistochemical features could be related to molecular status. We performed immunohistochemistry on 35 PSCGCs against TTF1, napsin A, p40, ALK, ROS1, and c-MET. Mutational status regarding EGFR, KRAS, BRAF, HER2, and PIK3CA genes was established. Of 18 PSCGCs with adenocarcinomatous or "undifferentiated" carcinoma differentiation, 8 were mutated for EGFR (n = 1), KRAS (n = 2), BRAF (n = 1), HER2 (n = 3), and PIK3CA (n = 1). No PSCGC (0/4) with only squamous cell or adenosquamous (0/2) differentiation was mutated. c-MET overexpression was only seen in PSCGC with adenocarcinomatous or undifferentiated component (n = 5) without squamous cell component. ROS1 and ALK were negative. The presence of a "targetable mutation" was correlated to the presence of morphological or immunohistochemical adenocarcinomatous differentiation (P = .0137). Integrated diagnosis of an adenocarcinomatous component in PSCGC could be associated with the presence of targetable gene mutation. Because only PSCGC with adenocarcinomatous or undifferentiated carcinoma harbors mutations, whereas PSCGC with only squamous or adenosquamous differentiation does not in our study, this might represent a prescreening for patients with PSCGC to be tested for molecular targets. Our results emphasize that careful morphological examination and the use of immunohistochemistry might be useful for the selection of PSCGC tested for a mutational target.

Primary giant cell carcinomas of the lung: a clinicopathological and immunohistochemical analysis of seven cases.

AIMS: Seven cases of primary giant cell carcinomas of the lung are presented. METHODS AND RESULTS: The patients were five women and two men between the ages of 48 and 72 years (average: 63 years). Clinically, the patients presented with symptoms of cough, chest pain, dyspnoea and general malaise. Diagnostic imaging revealed the presence of intrapulmonary masses; five tumours were located in the right lung and two in the left, with a general predilection for the upper lobes. All patients underwent surgical resection and staging of their tumours. Five patients were staged as T2 and T3 with nodal metastasis, while two patients in stages T1 and T3, respectively, had no nodal disease. Histologically, the giant cells were typed as syncytiotrophoblast-like or 'null type', according to the expression of ß-human chorionic gonadotrophin or expression of cytokeratin alone. Follow-up information revealed that five patients died within a period of 1-3 years, while two patients remain alive between 1 and 3 years. CONCLUSIONS: The cases presented herein highlight the importance of separating these tumours from the group of sarcomatoid carcinomas and analysing them carefully by immumohistochemical means, as we believe that these neoplasms represent a specific tumour entity.

Clinical analysis of 95 cases of pulmonary sarcomatoid carcinoma.

OBJECTIVES: To collect data on the clinical characteristics, pathologic presentation, and prognosis of patients with pulmonary sarcomatoid carcinoma. METHODS: From September 24, 2008 to June 3, 2014, 95 patients were hospitalized at the Shanghai Chest Hospital for the treatment of pulmonary sarcomatoid carcinoma. We retrospectively collected patient gender, age, smoking history, time of initial diagnosis, diagnostic methods, tumor location, pathohistological subtype, tumor size, TNM stage, immunohistochemical results, subsequent treatments, and patient survival. RESULTS: Of the 95 patients included in this study, 80 were male and 15 were female. Median patient age was 64 years (range: 43-80 years). There were 29 cases of pleomorphic carcinoma, one case of giant cell carcinoma, six cases of spindle cell carcinoma, and six cases of carcinosarcoma. The other 53 cases were not subtyped. The median survival was 11.54 months (range: 0.9-100.9 months). 1-, 2-, 3-, and 5-year survival was 32%, 30%, 25%, and 21%, respectively. Univariate analysis showed that tumor size, stage, T1+T2 vs T3+T4 stage, N stage, and M stage were prognostic factors for survival. Multivariate regression analysis showed that T stage and lymph node metastases were independent prognostic factors. CONCLUSION: Pulmonary sarcomatoid carcinoma is an uncommon, aggressive cancer. T1+T2 vs T3+T4 stage and lymph node metastases were independent prognostic factors. Our results underscore the importance of early detection and early diagnosis. Effective treatments for this disease are lacking.

Multiple skin cancers in a single patient: Multiple pigmented Bowen's disease, giant basal cell carcinoma, squamous cell carcinoma.

Basal cell carcinoma (BCC) and squamous cell carcinoma are the most common type of nonmelanoma skin cancers (NMSCs). Bowen's disease (BD), a premalignant condition, has a marginal potential (3-5%) to progress to invasive carcinoma. We report here a rarest of a rare case of multiple pigmented BD with overlying squamous cell cancer along with a giant neglected BCC on the scalp of a 76-year-old man. The occurrence of multiple BD and NMSC in a single patient compelled us to explore the following hypothesis: (1) The multiple precancerous and cancerous lesions can be due to common etiopathogenesis. Chronic ultraviolet exposure, immunosupresssion, human papillomavirus infection, dietary factors, and environmental factors including arsenic exposure were probed in to. (2) There is evolution of precancerous lesions into a different type of cancers in different time frame. (3) The new cancerous lesions are subsequent cancers that developed after neglected untreated primary cancer.

Small bowel intussusception caused by multiple intestinal metastases from a giant cell carcinoma of the lung: a case report.

Small bowel obstruction (SBO) due to intussusception in adults is a rare condition. Diagnosis at the time of admission is usually challenging. More often than not, a bowel intussusception in adults is secondary to an organic condition, frequently malignancies. Therefore, a surgical approach is indicated most of the times. We report the case of a forty-nine years old lady presenting with a SBO secondary to small bowel metastases with two ileo-ileal intussusceptions, one of which was missed at initial surgical exploration. A giant cell carcinoma of the lung (GCCL) with small bowel metastases was diagnosed subsequently. The case is presented as well as a brief review of literature.

Tratamiento del tumor de células gigantes con curetaje e injerto con hidroxiapatita porosa coralina HAP-200® / Treatment of the giant cells tumor with curettage and graft of porous coral hydroxyapatite HAP-200®

Se realizó la presentación de un caso con un tumor de células gigantes en el extremo distal del fémur derecho a nivel del cóndilo femoral interno. Se retira quirúrgicamente, previo estudio, donde se confirmó el diagnóstico a través de la biopsia. En dicho proceder se efectuó un raspado extenso de la lesión respetando la superficie articular del cóndilo femoral, rellenando la cavidad con la hidroxiapatita HAP-200 ®, sin necesidad de apoyar dicho acto con una osteosíntesis interna o externa, sólo con una calza de yeso por 6 semanas. El seguimiento del paciente ha sido, hasta la fecha, de 6 años y no se ha reportado ninguna recidiva o metástasis, con una osteointegración positiva del biomaterial, lográndose la curación ósea y una función articular sin restricción(AU)

We presented a case of a giant cells tumor in the distal end of the right thigh at the level of the internal femoral condyle. Previously studied, the tumor was surgically retired, and the diagnosis was established through the biopsy. In that procedure we carried out a lesion extended scrape respecting the join surface of the femoral condyle, filling the cavity with hydroxyapatite HAP-200®, being unnecessary to support it with an internal or external osteosynthesis, only with plaster wedge for 6 weeks. The patient´s follow-up has lasted for 6 years up to day, and no recidivism or metastasis has been reported, with a biomaterial positive osteointegration, achieving the bone healing and a join functioning without restriction(AU)

Granulocyte colony-stimulating factor-producing undifferentiated carcinoma of the colon mimicking a pulmonary giant cell carcinoma: a case showing overexpression of CD44 along with highly proliferating nestin-positive tumor vessels.

Granulocyte colony-stimulating factor (G-CSF)-producing tumors are known for their aggressive behavior. Only four cases of G-CSF-producing colorectal carcinoma have been previously reported. Herein, we present a case of an undifferentiated carcinoma of the descending colon showing G-CSF production and giant cell carcinoma morphology in a 93-year-old woman. A tumor with a diameter of 80 mm was identified in the descending colon via computed tomography. Descending colectomy was performed involving the abdominal wall where tumor invasion was observed. The white blood cell count, which was elevated before resection, decreased to normal levels after intervention. However, local recurrence at the resected site was detected 39 days after surgery. Upon recurrence, increased white blood cell counts and serum G-CSF were seen. The patient died because of respiratory failure 98 days after colectomy. By using immunohistochemistry, G-CSF expression was detected in tumor cells in the resected specimen, along with overexpression of CD44 and highly proliferating nestin-positive tumor vessels. The poor clinical outcome of this patient is consistent with previous reports that the expression of these three molecules predict poor prognosis. While G-CSF can be a therapeutic target considering its auto/paracrine function to induce tumor growth via the G-CSF receptor, CD44 and nestin may also be possible candidate therapeutic targets. Further studies are required to assess the efficacy of treatments targeting these three molecules.

Diagnóstico y tratamiento de los tumores pulmonares neuroendocrinos / Diagnosis and Treatment of Neuroendocrine Lung Tumors

Los tumores neuroendocrinos pulmonares (TNP) abarcan un amplio espectro de tumores que incluyen los carcinoides típicos (CT) y atípicos (CA), el carcinoma neuroendocrino de células grandes (CNCG) y el carcinoma microcítico de pulmón (CMP). Aunque ninguna variedad puede considerarse benigna, los CA y CT tienen un potencial metastásico mucho menor, habitualmente se diagnostican en estadios tempranos y la mayoría son subsidiarios de tratamiento quirúrgico. Se dispone de varias pautas de quimioterapia (QT) en caso de recidiva o en estadios avanzados, aunque la evidencia científica es insuficiente. Los CNCG, encuadrados en la clasificación actual junto a los carcinomas de células grandes, tienen rasgos moleculares, conducta biológica y perfil de sensibilidad a la QT que los asemejan más a los CMP. Con frecuencia su diagnóstico anatomopatológico es difícil, pese al uso de técnicas de inmunohistoquímica, y pueden ser necesarias muestras quirúrgicas. Las pruebas diagnósticas a utilizar son similares a las empleadas en otros tumores de pulmón, con algunas diferencias en cuanto al trazador óptimo que se usa en la tomografía de emisión de positrones. La nueva clasificación TNM es de utilidad en la estadificación de estos tumores. El síndrome carcinoide, muy infrecuente en los TNP, puede dar síntomas de difícil control que requieren medicación especial con análogos de la somatostatina y otros fármacos. En general, y con la excepción del CMP, se necesitan nuevos ensayos que den respuesta a numerosos interrogantes sobre el mejor tratamiento a aplicar en cada estirpe y cada estadio

Pulmonary neuroendocrine tumors (PNT) encompass a broad spectrum of tumors including typical carcinoid (TC) and atypical (AC) tumors, large-cell neuroendocrine carcinoma (LCNEC) and small-cell lung cancer (SCLC). Although no variety can be considered benign, AC and TC have a much lower metastatic potential, are usually diagnosed in early stages, and most are candidates for surgical treatment. Several chemotherapy (CT) regimens are available in the case of recurrence or in advanced stages, although scientific evidence is insufficient. LCNEC, which is currently classified alongside large-cell carcinomas, have molecular features, biological behavior and CT sensitivity profile closely resembling SCLC. Pathological diagnosis is often difficult, despite the availability of immunohistochemical techniques, and surgical specimens may be necessary. The diagnostic tests used are similar to those used in other lung tumors, with some differences in the optimal tracer in positron emission tomography. The new TNM classification is useful for staging these tumors. Carcinoid syndrome, very rare in PNT, may cause symptoms that are difficult to control and requires special therapy with somatostatin analogs and other drugs. Overall, with the exception of SCLC, new trials are needed to provide a response to the many questions arising with regard to the best treatment in each lineage and each stage

Hipernefroma: presentación de un caso / Hypernefroma: case presentation

El adenocarcinoma renal representa del 85 por ciento al 90 por ciento de todos los tumores primitivos del riñón, y el 2 por ciento de todos los cánceres. Afecta especialmente a varones entre la quinta y sexta décadas. Es un tumor caracterizado por tener un comportamiento absolutamente caprichoso y simulador de múltiples enfermedades, por lo que con razón se le ha calificado como "el tumor del internista". Por su similaridad histológica con la celularidad suprarrenal se pensó que se originaba a partir de restos suprarrenales, de ahí su nombre de hipernefroma. El cuadro clínico generalmente se caracteriza por dolor en flanco, hipertensión arterial y hematuria. Se presenta un paciente de 63 años de edad, raza blanca, sexo masculino, con antecedente de ser fumador de más de 40 años, diabético desde hace 1 año que tiene como tratamiento glibenclamida (5 mg) una tableta desayuno y comida. Hace aproximadamente más de 7 meses presenta decaimiento, astenia, anorexia y pérdida marcada de peso; hace 30 días se notó un aumento de volumen en cuello en la región supraclavicular izquierda no dolorosa (AU)

Renal cell carcinoma represents from 85 percent to 90 percent of all primitive kidney tumors, and 2 percent of all cancers. It especially affects men between the fifth and sixth decades. It is a tumor characterized by quite capricious and Simulator behavior of multiple diseases, so it has described as "the internist's tumor". By their histological similarity to adrenal cellularity it was thought that it was originated from adrenal rest that is why it is call hypernephroma. The clinical profile is usually characterized by flank pain, hypertension and hematuria. A case of a patient 63 years of age, white, male, with a history of smoking for more than 40 years, diabetic from 1 year which is treated with glibenclamide (tab 5mg) 1 tablet in breakfast and lunch. From 7 months ago he is presenting decay, asthenia, anorexia and marked loss of weight, and 30 days ago he noticed an increase of volume in neck in the left supraclavicular region not painful (AU)

[Case of giant cell anaplastic ductal carcinoma of the pancreas].

A 56-year-old woman was admitted to our hospital with fever and systemic malaise. Abdominal computed tomography revealed an enhanced tumor of the pancreatic head, measuring 9cm in maximal diameter and containing a low-density area. Subtotal stomach-preserving pancreatoduodenectomy was performed. Hemorrhage and necrosis were evident within the tumor, and osteoclastic polynuclear giant cells were also identified. A diagnosis of giant cell anaplastic ductal carcinoma of the pancreas was made. The patient has been free from recurrence for 6 months since surgery.

Giant ulcerating squamous cell carcinoma arising from linear porokeratosis: a case study.

Linear porokeratosis is one of the infrequent variants of porokeratosis, a rare disorder of keratinization that may develop into several epidermal malignancies, among them squamous cell carcinoma. Clinical surveillance for malignancy is imperative, but in cases when large or many lesions are present, surgical removal of porokeratosis lesions would result in an unfavorable amount of scarring. A case of a large, nonhealing full-thickness ulcer caused by a giant ulcerating squamous cell carcinoma occurring within lesions of long-standing linear porokeratosis is reported in a 43-year-old woman with a recent diagnosis of ulcerative colitis (UC). Wide excision of the ulcer and plastic surgical reconstruction of the area were performed. PET scans did not show metastases, so her prognosis is good based on definitive excision of the tumor. Physicians should be aware of this cutaneous disease and the importance of annual follow-up for these patients to monitor for any lesion that exhibits clinical features concerning for malignancy.