Uncommon Presentation of Metastatic Squamous Cell Carcinoma of the Skin and Treatment Challenges.

BACKGROUND Squamous cell carcinoma is one of the most common keratinocytic skin cancers, the other being basal cell carcinoma. It is the second most common skin cancer after melanoma. Cutaneous squamous cell carcinoma is mostly a localized disease. The metastatic presentation is rare even in the presence of invasive disease. The metastatic potential depends on the presence of high-risk features at the time of diagnosis. Lung, liver, and bone are the frequent sites of metastasis. Local and locoregional disease undergoes excision with or without adjuvant radiation. However, we lack proper treatment paradigms for this metastatic disease. CASE REPORT We are reporting a case of an elderly female with a history of high-risk localized cutaneous squamous cell carcinoma treated with complete local excision and radiation presenting 5 years later with extensive disease to the lung and liver, abdominal nodes, and spinal fracture. The patient was not a candidate for chemotherapy due to kidney failure. On the basis of ongoing separate trials on different immunotherapies, she was started on nivolumab. CONCLUSIONS Treating metastatic cutaneous squamous cell carcinoma is a challenge considering the absence of phase III trials due to the rarity of this disease. Historically, platinum with or without 5-FU (fluorouracil), bleomycin, doxorubicin, and retinoic acid were used with variable responses. Data on epidermal growth factor receptor (EGFR) inhibitors on EGFR expressing tumors are available. However, even with the most recent reports on immunotherapy in patients with high programmed death-1 expression or high mutation burden, it is difficult to achieve good response.

The diagnosis and management of desmoplastic small round cell tumor: a review.

PURPOSE OF REVIEW: Desmoplastic small round cell tumor (DSRCT) is a rare disease of children, adolescents and young adults, which begins in the abdominal cavity. Less than 200 cases are reported in the world literature. Because of the rarity of this disease, little is known about optimal treatment. Patients may present with dozens to hundreds of tumors studding the peritoneal cavity. Despite this presentation, it is not primarily considered metastatic but multifocal. It can metastasize to the liver or lung. Chemotherapy, radiotherapy, and surgical approaches have not been standardized. Neoadjuvant chemotherapy often yields a partial response; however, tumors may remain surgically un-resectable. An aggressive approach to treatment is required to maximize long-term remission. This review is designed to outline the evidence-based multidisciplinary approach to DSRCT. RECENT FINDINGS: Complete surgical resection, including 1-2 mm implants, is necessary to achieve long-term disease control. Hyperthermic intraperitoneal chemotherapy using cisplatin has recently been found to be a low morbidity treatment option for DSRCT patients. Yttrium microspheres have been used successfully to treat liver metastasis from DSRCT. SUMMARY: DSRCT is a rare tumor that requires a multidisciplinary approach which includes aggressive surgical extirpation to provide long-term disease control.

Treatment recommendations for primary extradural meningiomas.

Primary extradural meningiomas (PEMs) represent about 2% of all meningiomas and are often encountered by non-neurosurgeons. These lesions typically present as enlarging, painless, benign masses that can be surgically cured. Imaging is critical for defining involvement of adjacent structures; however, diagnosis depends on classic histologic patterns. Treatment for benign PEMs (WHO I) consists of resection with wide margins, whereas adjuvant therapy after resection of atypical (WHO II) or malignant (WHO III) PEMs should be considered. By using the collective experience from our comprehensive cancer center, including neuro-oncologists, neuroradiologists, and neurosurgeons, in addition to a complete literature review, the authors have established treatment guidelines not previously reported. This manuscript describes key features of these challenging tumors to aid in diagnosis, presents the largest published review of all reported PEMs (n = 163), and provides salient treatment guidelines to surgeons unfamiliar with these challenging tumors.

Giant desmoid tumor of the abdominal wall associated with familial adenomatous polyposis.

In this paper a case of vast desmoid tumor of the abdominal wall associated with familial adenomatous polyposis is reported. Desmoid tumors represent a particular type of fibrous neoplasms with a higher prevalence in females. They are extremely rare in the sporadic form, while they are found in about 10% of patients affected by familial adenomatous polyposis. Despite their benign histology and the absence of metastatic potential, they can be considered as fibrosarcomas with a low level of malignancy because of their locally invasive nature. The treatment of choice is surgical excision, as wide as possible, aimed at preventing recurrences, which are frequent in this tumor type. The usefulness of complementary therapies such as radiotherapy, hormone or chemotherapy, is not entirely clear.

Intra-abdominal desmoplastic small round cell tumor (IDSRT).

Intra-abdominal desmoplastic small round cell tumor (IDSRT) is a rare neoplasm that develops in the abdominal cavity in young people. We experienced a 27-year-old man who visited with ascites of unknown cause. Compression of the colon was found by barium enema examination. On colonoscopic examination, diffuse white elevated lesions, about 5 mm in diameter, surrounded by rubedo, so-called aphthoid lesions, were also observed. IDSRT was diagnosed by biopsy at laparotomy, and chemotherapy with cyclophosphamide, etoposide, doxorubicin, and cisplatin was performed. The tumors shrank temporarily (partial response), but subsequently grew in size again. The patient died during the second course of chemotherapy after relapse. We present one case report, together with a review of the literature.

[Cystic lymphangiomas of the abdomen. CT and US findings]. / Linfangiomi cistici dell'addome. Rilievi TC e US.

Cystic lymphangiomas of the abdomen are an uncommon kind of hamartoma. Their diagnosis is often difficult and calls for an accurate differential diagnosis of this disease and such lesions as cystic pancreatic neoplasms, pseudocysts, hematomas, abscesses and urinomas. Eight cases of abdominal lymphangioma are reported, in patients ranging 35 to 68 years; all lesions were uni/multilocular containing serous fluid. Lymphangiomas were located in the retroperitoneal space (3 cases), in the mesenteric bed (3 cases), close to the left lumbar ureter (1 case), and on the left colon wall (1 case); the symptoms were little characteristic, mostly due to pressure on the adjacent organs. Both CT and US were able to detect and evaluate the lesions; in particular, CT provided also with exact topography. A small lymphangioma in the left colon was revealed only by barium enema. In 2 huge retroperitoneal lymphangiomas the diagnostic evaluation was improved by the use of CT-guided fine-needle biopsy; in patients with mesenteric lesions angiography was employed, also for surgical planning. Lymphography was never performed because CT and US proved the best diagnostic procedures. All patients underwent surgery, whose results confirmed the previous diagnosis, but for the patient with para-ureteral location the surgeon thought nephrectomy necessary.

[Cystic lymphangioma of the abdomen in an adult. Contribution of echography and computed x-ray tomography. Apropos of a case]. / Lymphangiome kystique de l'abdomen chez un adulte. Apport de l'échographie et de la tomodensitométrie. A propos d'un cas.

Authors present a case of cystic lymphangioma of the abdomen in an adult patient. Pre-operative investigations are based on ultrasound and computed tomography since plain films, excretory urograms and barium enema are less helpful. Opportunity of such a diagnosis in adult is not frequent.

Pitfalls in the diagnosis of gastrointestinal tuberculosis.

Gastrointestinal tuberculosis is difficult to diagnose since it mimics many other abdominal conditions and has protean manifestations. The disease can present as an abdominal mass, bowel perforation, Crohn's disease and dysentery. These presentations are discussed with representative cases to illustrate the diagnostic difficulties. It is emphasized that to obviate the diagnostic pitfalls, tuberculosis should always be considered in the differential diagnosis of unusual gastrointestinal presentations particularly because radiological and laboratory tests may be noncontributory.

The value of laparoscopy under local anaesthesia in 250 medical and surgical patients.

This study reports our experience of laparoscopy under local anaesthesia in 250 patients; the procedure was safe, quick and easy to perform with minimal inconvenience to the patient. We advocate the early use of laparoscopy in patients with ascites and when there is difficulty in the diagnosis of patients with jaundice and hepatomegaly, an accurate histological diagnosis being obtained in the majority. In patients with intra-abdominal malignancy, in whom surgery is planned, laparoscopy can detect disseminated disease and so avoid an unnecessary laparotomy. Laparoscopy may also provide a diagnosis in patients presenting with a variety of vague symptoms such as abdominal pain, weight loss, lethargy etc. We have found laparoscopy an excellent investigation if positive but, as if only allows the surface inspection of viscera, when negative, we recommend caution in its interpretation.

The use of ultrasound to diagnose a pseudomass effect secondary to abdominal ascites.

Two cases of abdominal ascites in which a mass lesion was simulated on the roentgenogram are reported. Sonography permits ready differentiation of an encapsulated from a nonencapsulated fluid collection.

The value of laparoscopy in general surgical problems.

Laparoscopy offers a simple, rapid and safe way to evaluate and diagnose definitively intra-abdominal disease. An analysis of 65 consecutive patients supports this contention and demonstrates the real advantages and safety of this readily available modaility. A 90 per cent success rate, defined as accomplishing the diagnosis, was achieved in this study. There were no complications and no deaths. Local anesthesia was almost exclusively used and is strongly recommended. Laparoscopy represents an underused diagnostic modality which, when properly performed, can effect significant medical and fiscal economies in patient care.